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559 Cards in this Set
- Front
- Back
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most useful test to classify anemia
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MCV - Mean Corpuscular Volume --> measures volume of RBC
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common cause of Macrocytic anemia
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Vit B12 or Folate deficiency
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MCV = 80 um3
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Microcytic anemia
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MCV = 80 - 100 um3
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normocytic anemia
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MVC > 100 um3
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Macrocytic anemia
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RDW
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reflects variation in size of RBC in periperial blood. -inc when rbc not same size
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cause of microcytic anemia with inc RDW
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mix of normocytic and microcytic RBC
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location of iron absorption
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duodenum
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location of folate absorption
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jejunum
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location of Vit B12 absorption
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terminal Ileum
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diseases see target cells
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Alcholics ->alter chol in membrane hemoglobinopathy -thalasemia -sickle cell -HbC
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key characteristic seen in microcytic anemia in smear of peripheral blood
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rbc with inc palor due dec in Hgb synthesis
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MCHC
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correlates with presence of spherical rbc -> spherocyte
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anorexic cell with too little membrane and no central are of palor
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spherocytes
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test use to decide how to work up normocytic anemia
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reticulocyte count
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equation for corrected reticulocyte count
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(actual Hct (%) / 45) x % reticulocyte count -->divid by 2 final answer if have polychromasia
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> 3% corrected reticulocyte count
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bone marrow responding properly
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<2% corrected reticulocyte count
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bad / poor bone marrow response
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mechanism of microcytic anemia
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cant make Hgb
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most common cause of sideroblastic anemia
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chonic alcoholism
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alcohol effect on Hgb synthesis
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etoh mitochondrial toxin damage heme biosyn pathway in mitochondria develop Fe overload bc Fe trapped in mitochondria and develop ringed sideroblasts in bone marrow
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rate limiting enzyme in heme syn
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ALA synthase
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component of Hgb
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Globin: alpha beta delta gamma Heme -> Fe and protoporphyrin IX
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cause of fe deficiency in chronic infection
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since Fe stored in macrophage of bm body locks up Fe from both bacteria causing infection and RBC
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directly controls RBC development in BM
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Hgb -determines # of cell divisions
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Dec HgB-> ________ RBC divisions
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inc -> microcytic anemia
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necessary enzyme and cofactors to convert Succinyl-CoA in Heme syn
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succinyl Co-A converted to ALA using 1. Glycine ->inhibitory NT -blocked by botulin toxin causes spasms 2. Vit B6 (Pyridoxine) 3. ALA Synthetase - rate limiting enzyme
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heme enzyme most denatured by Pb and result
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ferrochelatase -enzyme used to bring protporphyrin IX together with Iron to form Heme Without active/functional ferrochelatase: -Fe cant bind with protoporphyrin to form heme
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inc heme dec ________
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ALA synthetase neg feed back of heme syn
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thalamssemia in southeast asian and blacks
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alpha- thalassemia
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autosomal recessive type of anemia
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Thalassemia - problem in forming alpha globin chains
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primarly used to detect hemoglobinopathies
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Hb electrophoresis -seperate by size and charge -seperates: Hb A Hb F and Hb A2
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most common Hb detected in Hb electrophoresis
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HbA -> has 2 alpha globin chains and 2 beta globin chains
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cause of alpha thalassemia
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gene deletion
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alpha thalasemia minor
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2 gene deletion out of the 4 genes that control alpha-globin chain synthesis -mild anemia -microcytic with dec HbG as catalyst for inc mitosis -in black and asian pop
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HbH
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3 of 4 gene deletion for formation of alpha globin chain -type of alpha thalassemia -severe anemia -HbB formed when 4 beta chains combine -detected by electrophoresis
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Hb Bart
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4 of 4 gene deletion for alpha globin chain -4 gamma globin chains combine -not compatable with life -results in spontateous abortions -if inc in spontaneous abortions then inc in chorionic carcinoma esp in asian pop
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alpha thalasemia is tx with Fe t or f
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False this will cause Fe overload and kill pt leave pt alone
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pop with Beta Thalassemia
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black greek asian
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mild B-Thalassemia
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DNA splicing defect
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severe B-Thalassemia
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nonsense mutation with formation of stop codon
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2 alpha/ 2 Beta globin chain stucture
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HbA
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2 alpha/ 2 gamma globin chains
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HbF
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2 alpha/ 2 delta globin chains
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HbA2
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Dec in HbA inc HbA2 and HbF inc RBC count
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Beta - thalassemia minor (B/B+)
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what test dx B-Thalassemia
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Hb Electrophoresis
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Cooley's anemia
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B-thal major (Bo/ Bo) -severe hemolytic anemia -rbc with a-chain inclusion removed by macrophage in spleen -cause inc in uncong bilirubin
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type of microcytic anemia requiring long term blood transfusion and at risk for Fe overload
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Beta - Thalassemia Major (Bo/Bo)
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type of anemia caused by any of following chronic alcoholism pyridoxine deficiency lead poisoning
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sideroblasitic anemia
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most common cause of anemia in women < 50
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menorrhagia
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most common cause of anemia in men < 50
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pud
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most common cause of anemia in adults > 50
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polyps / colorectal cancer
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dec serum ferritin
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dx of Fe deficiency
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primary soluble Fe storage protien
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ferritin - serum levels correlate with Ferritin stores in macrophages -
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calculate iron saturation (%)
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= serum Fe / TIBC x 100 TICB = serum total iron binding capacity = transferrin which carries Fe
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dec % Fe saturation
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Fe deficiency ACD
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dec TIBC
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ACD
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inc serum ferritin
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ACD Fe overload disease
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inc serum Fe
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Iron overload disease
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inc % Fe saturation
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Fe overload disease
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lab test used to distinguish between ACD and anemia due to Fe deficiency
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serum ferritin bc ferritin is the primary soluble fe storage protien stored in BM macrophages so if Fe is locked in the BM macrophages by the body so that Fe does not get to bacteria in a chronic infection then with ACD inc in ferritin
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What microcytic anemia (s) has normal iron studies?
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alpha and beta thalassemia because have to do with globin chain not Fe
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microcytic anemia dx by BM biopsy
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siderblastic anemia
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coarse basophilic stippling of RBC
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lead poisoning -Pb denatures ribonuclease so ribosomes can not be degraded and persist in the RBC -seen in peripheral blood
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persistant ribosomes in RBC
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seen as course basophilic stippling caused by Pb poisoning
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stain used to id sideroblastic anemia
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prusian blue
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RNA filament
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reticulocyte
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deposit in ephysis of bone
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Pb - cause dec in growth
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child presents with abdominal cholic cerebral edema convulsions severe microcytic anemia
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Pb poisoning
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MOA of cerebral edema (encephalopathy) in children
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delta - ALA damages neurons increases vessel permeability (cerebral edema) and causes demyelation
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Auto worker presents Cholic Diahrea
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Pb poisioning
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Man who makes moonshine presents with abdominal colic with diarrhea
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Pb poisoning
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slapping gate radial and ulner nerve palsy claw hand wrist drop peritonal palasy
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neuropathy seen in adults with Pb Poisoning
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Fe overload disease associated most with __________. serum Fe TIBC Serum Ferritin % Fe saturation
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Pb poisoning serum Fe -> inc TIBC -> dec -why bc when inc in ferritin which stores Fe then transferritin (TIBC) which binds to ferritin dec serum ferritin -> inc % Fe saturation -> inc
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cant make dTMP
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-lack both folic acid (tetrahydrofolate) and Vit B 12 (cobolamin) - results in immature large nuclei- less dna- so huge nucleated cell -> Macrocytic Anemia as make more dna- cell matures and dec in size
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cobalamin
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Vit B12
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methyl tetra hydrofolate
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circulating folate in blood
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purpose of cobalamin
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take methyl group off Methy tetrahyrdofolate
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high levels of homocysteine
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-seen with folate and Vit B12 deficiency -leads to thombi and mi -homocysteine damages endothelial cells which induces thrombosis and MI
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most common cause of elevated homocycteine levels
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folate deficiency
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2 compounds needed to make DNA and key enzyme
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folate (Methyl Tetrahydrofolate) and Vit B12 (cobolamin) need thymidylate synthase
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causes dementia and proprioception
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Vit B 12 deficiency -involved in odd-chain fatty acid metabolism
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cofactor need for converting methylmalonyl CoA to Succinyl CoA
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Vit B 12
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sensitive and specific test for Vit B 12 deficiency
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methylmalonic acid levels inc
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cause of dementia demilination of posterior colum and lateral spinal tracts
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due to lack of Vit B 12 -no propionic metabolism so now succinyl CoA produced and no mylien produced
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neurologic problems due to Vit B12 defiency
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problems with vibration proprioception spasticity abn babinski sign dementia Not involved with Folate Deficiency can not correct with folate correct only with Vit B 12
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reversible causes of dementia
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Vit B 12 deficiency TSH deficiency - to rule out hypothryoidism
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R-Factor (R-binder)
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binds Vit B 12 in saliva to prevent the Vit B 12 from being destroyed in stomach acid
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intrisic factor (IF)
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produced by parietal cells in body fundus of stomach delivers Vit B12 to terminal ileum
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cleaves off r-factor
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pancreatic enzymes releases Vit B 12 to bind to IF
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chron's disease
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have bile salt defiency and Vit B 12 diffiency because both are absorbed at the terminal ileum and chrons diease effects the terminal ileum
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autoimmune disease against paritial cells
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pernicious anemia
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most common cause of Vit B 12 deficency
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pernicious anemia
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predisposure of gastric adenocarcinoma
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agastritis due to pernious anemia and distruction of parietal cells in the body and fundus of the stomach
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causes of Vit B 12 deficiency
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-pernious anemia -pure vegans -chronic pancretitis - chronic alcoholics -fish tape worm -lack of peristalsis of segment of intestine ->bacterial overgrowth - love B 12 - IF and bile salt -crohn's disease
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intestinal conjugase
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enzyme which converts the folate polyglutmate form to a monoglutamate form
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phenytoin
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blocks or inhibits actions of intestial conjugase
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pt presents macrocytic anemia with hypersegmented neutrophils with normal neurologic exam- what drug is the pt on?
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phenytoin
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location in SI where folate absorbed
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jejunum
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most common cause of folate deficiency
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alcoholism
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______________ & ____________ inhibits monoglutamate reabsorption
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birth control pills and alcohol
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folate supply in body
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3 to 4 months
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neutrophil with greater than 5 segments
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seen with megaloblastic anemia - hypersegmented neutrophil marker of folate and vit B 12 deficiency if have neurological defiency -> vit B 12 def if No neurologic deficiency -> folate deficiency
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test for proprioception
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rhomberg test cant keep balance when close eyes
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pancytopenia oval macrophage segmented neutrophils of more than 5 lobes
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Vit B 12 deficiency -> megoblastic anemia
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Shilling test
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give pt radioactive B 12 and then collect 24 hr urine to measure vit B 12 reabsorption
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give pt IF then radioactive B 12 do shilling test see B 12 in urine disease
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pernicious anemia
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give pt antibiotics then radioactive B 12 do shilling test see B 12 in urine disease
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bacterial overgrowth detroying vit B 12 - IF complex
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give pt pancreatic extract then radioactive B 12 do shilling test see B 12 in urine disease
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chronic pancretitis lacking pancreatic enzymes to cleave off R- factor ( R-binder)
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acute blood loss < 5 to 7 days early Fe Deficiency ACD aplatic anemia chronic renal disease
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all cause nomocytic anemia with corrected reticulocyte count less than 3% note: takes 5 to 7 days before reticulocyte response
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develop _________anemia before ________________anemia with pt with early Fe defiency or ACD
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normocytic - microcytic
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what distinguishes btween early Fe deficency and ACD
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serum ferritin
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signs of volume depletion
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dec bp- inc pulse
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aplastic anemia
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no marrow - all hemopoietic cells in marrow destroyed pancytopenia in peripheral blood
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most common cause of aplastic anemia
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most cases idiopathic drugs most common known cause - indomethocin methylbutozon infections - Hep C common
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biggest infection causing aplastic anemia
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Hep C
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aplastic anemia of RBC only- all else normal- infection?
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parovirus
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most common cause of normocytic anemia with corrected reticulocyte count of less than 2% with chronic renal failure
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dec EPO - most common cause
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RBC phagocytosis by macrophage in spleen and liver
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extravascular hemolysis
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reason for extravascular hemolysis
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1. rbc coated with IgG w/ or w/o C3b 2. abnormal shaped rbc -> spherocyte- sickle cells
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inc serum unconjugated bilirubin and LDH
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due to extravascular hemolysis inc serum unconjugated bilirubin -> end product of macrophage degradation of Hb inc lactate dehydrogenase (LDH) from hemolyzed rbc
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how are spherocytes and sickle cells removed from circulation?
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by extravascular hemolysis
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autoimmune hemolytic anemias
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rbc coated by IgG with or without C3b -> removed by macrophages so extrinsic hemolysis
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clinical finding in pt with extravascular hemolytic anemia and why?
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jaundice- bc unconjugated bilirubin released from phagocytized rbc goes to liver to be conjugated -lipid soluble and bound to albumin so not excreated in urine
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hemolysis occur within bv
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intravascular hemolysis
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G6P deficiency IgM - mediated hemolysis (complement destruction) calcific aortic valve stenosis
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causes of intravascular hemolysis
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inc plasma and urine Hb
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due to intravascular hemolysis
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haptoglobin
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protien produced by liver to bind to free Hb
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dec serum haptoglobin and hemoglobinuria
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intravascular hemolysis
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defect in rbc - such as membrane defect - no spectrin in cell membrane abnormal Hb - HbS enzyme defiency - no G6P in pentose phosphate shunt causing anemia
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intrinsic hemolytic anemia
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factors out side rbc- such as stenotic aortic valuve immune destruction - IgG and C3b on rbc causing hemolysis
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extrinsic hemolytic anemia
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autosomal dominant disorder with intrinsic defect result in extravascular hemolysis
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hereditary spherocytosis
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mutation in ankyrin in cell membrane- also mutations in band 2- spectrin ( a and b)- or band 3 account for other defects
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hereditary spherocytosis
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dx test for herditary spherocytosis
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inc rbc osmotic fragility -inc permeability of spherocyte to Na and water -spherocytes rupture in mild hypotonic salt solutions
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treat with splenectomy
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hereditary spherocytosis so spherocytes remain in peripherial blood
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acquired membrane defect in multipotent myeloid stem cells -mutation causes loss of the anchor for decay accelerating factors (DAF) nomocytic anemia with pancytopenia
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PNH - paroxysmal noctural hemoglobinuria
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screened using sucrose hemolysis test confirm using acidified serum test
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PNH
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HbS > 60% and inc oxygen tention (inc deoxy Hb)
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causes sickling
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genetics of sickle cell anemia
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autosomal recessive disorder 25% normal 50% trait 25% actual disease
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no anemia- microhematuria
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do sickle cell screen for sickle trait
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extravascular hemolytic anemia and microvascular occlusions
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sickle cell anemia -result in pain due to ischemia caused by the microvascular occusions in any organ - long term damage to organ in children pain start in hands and feet
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Howell-Jolly bodies
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nuclear remnants which appear in RBC indicating loss of macrophage fxn in spleen- spleen is enlarged and dysfxn (autosplenectomy)occur by 2 yr old child with homozygous sickle cell anemia (HbSS)
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most common cause of death in a child with sickle cell anemia
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streptococcus pneumoniae sepsis
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pneumovax
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vaccine given to children at 2 years old- especially helpful in sickle cell anemia pt
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painful swelling of hands and feet in infant (6 to 9 mo) due to bone infarcts
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dactylitis - clinicall finding of HbSS - sickle cell anemia occur at 6 mo bec HbF prevents sickling of Hb- so as HbF dec and HbSS inc start with pain
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osteomyelitis in sickle cell anemia is due to ____________
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salmonella paratyphi
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anemia which is x-linked recessive
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G6PD
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Name 2 sex linked recieve enzyme deficiencies
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1. G6PD 2. Lesch-nyhan syndrome - ALWAYS ON BOARDS -involves purine metabolism -> HPRT deficient
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intrinsic defect with perdominantly intravascular hemolysis
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G6PD
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leads to dec syn of NADPH and glutathione (GSH)
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in the pentose phosphate pathway
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why are Hb oxidized in G6PD
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G6P usually help produce NADPH and glutathione (GSH) GSH usually neutralized hydrogen peroxide- but with defiency of G6P- excess peroxide oxides Hb- which preceptiates to form Heinz bodies
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ptdevelops malaria and is given drug and after 2 - 3 days develops the following: hemoglobinuria chills hemolytic anemina sudden onset of back pain What drug was given
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any of the following primaquine dapsone - treats leprosy chloroquine sulfonamides
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Heinz bodies
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oxidized Hb that is clumped up
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bite cells
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heinz bodies removed from rbc by spleen macrophage producing bite cells in pt with G6PD
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Oxidant damage with heinz bodies and bite cell
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G6PD
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test used after hemolytic episode is over- confirm dx
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G6PD assay
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primarly causes intravascular hemolysis due to oxidized Hb
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G6PD
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enzyme assay is done before after or never when a hemolytic episode is occuring in a G6PD pt
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after because the assay will show normal during the hemolytic assay bc the cells with the enzyme survived- the cells without the enzyme have been hemolyzed. During a hemolytic episode- stain for Heinz bodies.
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warm reacting Ab
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IgG
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cold reacting Ab
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IgM
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autoimmune warm type most common cause of this anemia
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immune hemolytic anemia
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most common cause of autoimmune hemolytic anemia (AIHA)
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SLE
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if a family has a history of autoimmune diseases which lab test is best for screening?
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HLA- because often times if pt has one autoimmune disease they can develop another- the same is true for other family members
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rbc coated with IgG are phagocytosed by splenic marcorphages
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IgG - mediated hemolysis- occurs by extravascular hemolysis spherocytes formed if small part of membrane removed
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clinical findings of what disease 1. jaundice due to unconjugated hyperbilirubinemia due to extravascular hemolysis 2. hepatosplenomegaly due to hyperplasia of splenic and liver macrophages 3. raynaud's phenomenon
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Autoimmune hemolytic anemia with #3- can be cold type AIHA
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Coomb's test
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lab test used to evaluate autoimmune hemolytic anemia also refered to as DAT = direct antihuman globulin test can be direct or indirect
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detects RBC sensitized with IgG and/ or C3b
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dirct antihuman globulin test (DAT) or Coomb's test
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detects antibodies inthe serum- ex -> anti-D antibodies
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indirct antihuman globulin test or indirect Coomb's test
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drug which causes body to develop antibodies against it- the Antibodies attache directly to the drug which is attached to the RBC membrane
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penicillin
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type of hypersensitivity caused by penicillin resulting in hemolytic anemia
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Type 2
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pt on penicillin and develops a rash- what type of hypersensitivity?
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type 1
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2 possible drugs which can be given to pregnant women for htn
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methyl dopa and hydralazine
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se of hydralazine
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drug induced lupus
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qudrug acts as haptan and IgM binds to drug to form immune complex 1. what drug? 2. what type of hypersenstivity forms 3. what type of anemia reults
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1. quinidine 2. type 3 3. intravascular hemolytic anemia which dec haptoglobin protien and Hb in urine
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schistocytes
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sign of microangiopathic hemolytic anemia (MHA) -fragmented rbc with absence of central pallor -produces when rbc mechanically injured by calcium deposits in an aortic valve- platelet thrombi or fibrin clot in microvasculature
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platelet thrombi (HUS) or fibrin thrombi (DIC) can cause this type of anemia
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microangiopathic anemia
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aortic stenosis (valvular defects) is the most common cause of this type of anemia
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macroangiopathic hemolytic anemia
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fragmented rbc caused by microcirculartion lession with dec MVC in pt with valvular defect
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microangiopathic hemolytic anemia related to aortic stenosis
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hemolytic uremic syndrome (HUS) thromboticc throbocytopenic purpura disseminated intravascular coagulation (DIC)
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all three can be a cause of microangiopathic hemolytic anemia and see schistocytes as a sign
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occur to runners
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runner's anemia bc rbc crushed in sole of feet when running on hard pavement damage rbc so cause hemoglobinura
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see rings form inside rbc
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p. falciparum malaria- see superinfection with multiple rings
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causes intravascular hemolytic anemia with fever spikes
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malaria
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most lethal type of malaria with ___________fever pattern which spikes with no pattern
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plasmodium falciparum- quotidian fever pattern
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benign exagerated leukocyte response to infection such as TB or sepsis esp seen in children but can also be seen in adults
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leukemoid rxn
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can be caused by infections -mononucleosis - due to epstein bar virus infecting b cell attaching to receptor CH 21 -hepatitis virus -cytomegalovirus (CMV) -toxoplasmosis also caused by drugs including phenytoin
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atypical lymphocytosis
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in slide see -cell with prominent nucleoli and coarse nuclear chromatin -cytoplasm is abundant and is indented by adjacent rbc
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atypical lymphocytosis
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caused by epstein bar virus (EBV) -transmitted by kissing -found dormate in _______cell can also attache to ___________ on this cell
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Infectious mononucleiosis B cells CD 21 receptor
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clinical findings of what condition? fatigue tonsillitis hepatosplenomegaly and generalized lymphadenopathy -danger of splenic rupture in contact sports rash develops if tx with ampicillin
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infectious mononucleosis
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antigenically stimulated lymphocyte prominent nucleoli and abundant blue cytoplasm
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atypical lymphocytosis
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15 yo male presents with tonsillitis fatigue hepatosplenomegally as well as generalized lymphanopathy what test should be ordered to confirm the dx
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positive heterophile antibody test commercially known as mono spot test
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test that detects IgM antibodies against horse (most common)- sheep- bovine rbc
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hetrophile antibody- test for mononucleosis
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atypical lymphocytes are antigenically __cells which respond to ____ cells infected with EBV. FILL IN BLANKS AND NAME DISEASE
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True B- infectious mononucleosis
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cell type seen in chronic infection
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monocytosis
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diseases which have monocytosis (monocyte count > 800uL)
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RA - autoimmune diseases TB - chronic infections carcinomas / malignant lymphomas - malignancies
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T or F pinworm and adult ascariasis do have eosinophila
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f bc these are non invasive pinworm - mate and lay eggs in anus adult ascariasis- get together and obstruct your bowel - larva cross lung is invasive
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strongyloidiasis and hookwork infection
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invasive helminthic infection one of the causes of eosinophila
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T or F protozoans cause eosinophila
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F
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T or F Bronchial asthma - reaction to penicillin - hayfever do not cause eosinophila
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f They do because these are type I hypersensitivity
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polycythemia vera
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basophila -it is a chronic myeloproliferative disorder 2 categories relative most commonly caused by dec plasma vol so inc rbc count and normal rbc mass appropriate with hypoxia to see inc rbc also with tumor producing epo chronic myeloproliferative disorder can lead to leukemia
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basophila prominent in this disease
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chronic myeloproliferative diseases
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leukemia in 0 to 14 years old
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acute lymphoblatic leukemia - ALL CD10 and TdT (terminal dioxynucleotidyl transferase) positive
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leukemia in 15 to 39 years old
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AML - acute myelogenous leukemia see Myeloblast with Auer rods
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leukemia in 40 to 59 years old
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AML - acute myelogenous leukemia - > 30% blast count CML - chronic myelogenous leukemia - < 30% blast count
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leukemia in > 60 years old
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CLL - chronic lymphocytic leukemia
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most common overall leukemia
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CLL - chronic lymphocytic leukemia
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most common cause of generalized lymphadenopathy in people > 60 years old
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CLL - chronic lymphocytic leukemia
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t9;22 translocation of ABL proto-oncogene -proto - oncogene fuses with the break cluster region (BCR) on chr 22 (BCR-ABL Fusion gene)
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CML - Chronic Myelogenous leukemia
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2 tests to prove CML
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1. phiadelphia chromosome study 2. leukocyte alkalin phosphate score - usually low because neoplatic granules do not stain
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most sensitive and specific test for CML
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BCR-ABL fusion gene
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-fibrosis of bone marrow -neoplastic cells produced in spleen result in massive splenomegaly with portal hypertension -tear drop rbc
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myelofibrosis andmyeloid metaplasia
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CALLA - CD10 and TdT positive
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ALL
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62 old man with splenomegaly and no lymphadenopathy and positive TRAP (tartrate - resistant acid phosphatase stain)
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Hairy cell leukemia
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35 year old pt with 5000 wbc count - many abnormal cells with rod shaped structures fused with azurophilic granules in cytosol of myeloblasts also anemia and thrombocytopenia also 70% blast in bone marrow
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AML - acute myelogenous leukemia
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leukemia with no auer rods and likes to inflitrate gums
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acute monocytic leukemia
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what type of leukemia has many auer rods- always presents with DIC (diseminated intervascular coagulation)- t15;17 translocation and can be tx with vit A which may induce remission by maturing cells
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acute promyelocytic leukemia - M3
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leukemia with t15:17 translocation
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acute promyelocytic anemia
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leukemia treated with retinoic acid (Vit A)
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acute promyelocytic leukemia
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neoplastic cell of Hodgkin's lymphoma
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Reed-sternberg cells
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most common lymphoma in children in the usa seen an abdominal nodes and even testes
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burkitt's lymphaoma
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lymphoma derived from germinal center causes t(14;18) causing overexpression of BCL2 antiapoptosis gene
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follicular lymphoma
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what 2 tissues are resistance to malignancy invasion?
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cartilage and elastic tissue
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lymphoma with EBV relationship with t(8:14) - mic oncogene from chr 8 Starry sky apperance with neoplatic B cells (dark sky) and macrophages (stars)
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burkitt's lymphoma
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type of lymphoma that manifests in the jaw
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african burkitt's lymphoma
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neoplasm of CD4 T helper cells involving skin with rash to plaques to nodular masses
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mycosis fungoides -can also include lymph nodes lung and liver and spleen
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lymphoma that involves malignant T helper cells in circulation
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Sezary syndrome - only difference from mycosis fungoides is that it is the malignant T helper cells are in circulation
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painful lymphadenopathy caused by?
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inflammation ie infection
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examples of localized lymphadenopathy
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tonsillitis - drain sites of infection anterior cervical notes and ingual nodes most common sites
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painful lymphadenopathy with a pt that has SLE
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generalized
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type of lympadenopathy? painful can be systemic can be seen in pt with mono (EBV) and SLE and HIV
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generalized painful benign lymphadenopathy
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painless adenopathy
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malignancy
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axiallary nodes in breast cancer which are painless
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localized malignant lympadenopathy
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metastasis in leukemia or follicular B-cell lymphoma
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generalized malignant (painless) lymphadenopathy
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3 parts of lymph node and what is produced in each
|
germinal follicle - macrophage brings antigens here to B cells which devellop into plasma cells and releast Ab Sinus - involves histocytosis X paracortex - T cells located here
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lymphadenopathy seen in paracortex or paratrabecular area of lymph node
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Di Georges Syndrome
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lymphadenopathy involving sinuses of lymph nodes
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metastasis - sinus histocytosis - Langerhans cells histocytosis (histocytosis X) - histocytic lymphoma
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reactive lymphadenitits and follicular B-cell lymphoma are found in what part of lymph node
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germinal follicle
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Hodgkin's lymphoma is catergorized as ________ _______ lymphadenopathy
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localized painless
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Metastasis in leukemia and follicular B-cell lymphoma are both catergorized as __________ __________ lymphadenopathy
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generalized painless lymphadenopathy
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virus involved with burkitt's lymphoma
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EBV
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consist of CD 1 positive and contain Birbeck granules that have a tennis racket apperance
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langerhan histocytes
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diffuse eczematous rash in a child with CD1 and tennis racket apperance granule
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Letterer-Siwe disease - malignant histocytosis
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bacteria that appears as a tennis racket shape but does not stain and has terminal spores
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clostridum tetani
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4 H's: hyperviscosity causing inc thrombosis hypervolumia histamina hyeruricemia pt taking shower in cold or hot water and reports itching
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polycythermia
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neoplastic stem cell disorder
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myeloid disorders
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inc rbc count dec PV (plasma volume) normal RBC mass and SaO2 and EPO example is volume depeletion from sweating
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relative polycythemia
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inc rbc mass and EPO normal PV (plasma volume) dec SaO2 - seen in COPD and people who live in high altitudes and cyantoic congential heart disease such as Tetrology of Fallot
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Appropriate polycythemia
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inc RBC mass - inc PV (plasma volume) - normal SaO2 - dec EPO
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polycythemia vera
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clonal expansion of trlinage myeloid stem cells - inc rbc - inc granulocytes ( neutrophils and eosinophils and basophils) and mast cells and platelets
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polycythemia vera
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pt presents with splenomegally - thrombotic event due to hyperviscosity and signs of increase histamine release from mast cells in skin expressed as ruddy face - pruritis after bath and peptic ulsce disease due to histamine stimulating gastric acid production
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polycythemia vera
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RBC mass inc - normal PV - SaO2 normal - inc EPO (why)? what type of polycythemia
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inappropriate polycythemia ecotopic EPO - why inc in EPO? bc EPO is being ectopically produced - seen in renal disease and renal carcinomas
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t 9:22 translocagtion of ABL proto-oncogene which fuses iwht break region (BCR) on chr 22
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CML
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normocytic anemia with tear drop shaped rbc
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myelofibrosis and myeloid metaplasia
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marrow fibrosis occurs early and neoplastic cells are produced in the spleen leading to massive splenomegaly and tear drop rbc
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myelofibrosis and myeloid metaplasia
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spliner shaped rodes torode shaped structurs in cytosol of myeloblasts and fuse to azurophilc granules also considered abnormal lysosomes
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Auer rods
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name disease 1. malignant stem cells in marrow - metasisis anywhere - always has generalized lymphadenopathy and often see hepatomegaly 2. abnormal cells in peripherial blood refered to as Blasts 3. always has anemia 4. thrombocytopenia 5. abnormal WBC count
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leukemia
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patient presents with: generalized lymphadenopathy - metastatic sites - inc incidence of both warm and cold types of hemolytic anemia
|
CLL - chronic lymphocytic leukemia
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type of leukemia where hypogammaglobulinemia is common
|
CLL - chronic lymphocytic anemia
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leukemia with < 30% blasts and has philidelphia chromosomes
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CML
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most common cancer of lymph nodes
|
non Hodgkin's lymphoma
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follicular B cell lymphoma with t(14: 15) translocation where p50 apoptosis gene is removed and cell immortal
|
Non-Hodgkin's Lymphoas (NHL)
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most common type of Hodgkin's lymphoma
|
Nodular Sclerosing Hodgkin's lymphoma
|
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type of lymphoma seen in women most of the time and involves anterior mediastinal nodes and either cervical or supraclavicular nodes
|
Nodular Sclerosing Hodgkin's Lymphoma
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lymphoma with the malignant cell -> Reed - Sternberg (RS) cells
|
Nodular sclerosing Hodgkin's Lymphoma
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polyclonal gammopathy
|
category of lymphona that is benign and due to chronic inflamation
|
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monoclonal gammaopathy
|
malignant - 1 clone of plasma cell making immunoglobulin most likely IgG and immunologically supressing the others. Produces lots of light chains excreted in the urine called bence jones protiens
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type of lymphoid tissue disease known to have Bence - Jones protiens in the urine also see M - spike (monoclonal) of IgG 80 to 90 % of the time Punched out lytic lesions are seen esp in the vertebra
|
Multiple Myeloma
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light chains excreted in urine
|
bence jones protien
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|
What are the bone findings of Multiple Myeloma?
|
lytic lesions - pathologic fractures - hypercalcemia
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|
eldery women that coughs and has sever pain in ribs. goes for x-ray and pathologic fractures seen along with lytic lesions. What is the disease?
|
multiple myeloma
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cell type that had bright blue cytoplasm with clear area next to cytoplasm consisting of RER
|
plasma cell
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light chain are converted to amyloid and produce a nephrotic syndrome
|
primary amyloidosis associated with multiple myeloma
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|
pt dies at 40 - following an autopsy where brain is atropy and senile plaques are seen in the frontal and temporal lobs
|
pt has down syndrom with trisomy 21 which produces B-amyloids which are toxic to neurons
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|
anticoagulation compounds naturally found in the body
|
1. heparine-like molecules - enhance antithrombin III which neutralized serine proteases coagulation factors (F12/11/9/prothrobin - F2 /and thrombin 2. prostaglandin I2 - produced by intacted endothelial cells to cause vasodilation and inhibit platelet aggregation 3. Protien C and S - Vit K dependent factor - inactivates factor 5 and 7 4. tissue plasminogen activator (tPA) - release plasmin which lyses fibrin clots (thrombi)
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enhances ATIII
|
heparin
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vasodilator and inhibits platelet aggregation
|
PGI2
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inactivates factors V and VII and enhances fibrolysis
|
protien C and S
|
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activates plasminogen to release plasmin
|
tPA
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|
why birthcontrol pills thrombogenic
|
inc syn of factors V and VIII - inc fibrinogen - inhibits ATIII antagonist to thrombi
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|
procoagulant released in small vessel injury
|
thromboxane A2 - vWF - tissue thromboplastin (Factor III)
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|
|
-made by megakaryocytes in bm and endotheial cells -platelet adhesion and prevent degradation of VIII :C
|
vWF
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|
what binds to platelet receptor GpIb
|
vWF
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|
|
What platelet receptor binds to fibrinogen
|
Gp IIb/IIIa
|
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|
what chemical released from the dense bodies of platelets aggregates platelets
|
ADP
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|
|
3 fxn of thromboxane A2
|
1. potent vasoconstrictor 2. bronchoconstrictor 3. platelet aggregate
|
|
|
factor VII
|
extrinsic factor
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|
factors XII and XI & IX & VII are what part of the coagulation pathway
|
intrinsic pathway
|
|
|
what factors make up the common pathway of the coagulation pathway?
|
factors X & V & II (prothrombin) & I (fibrinogen)
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|
|
what crosslinks insoluble fibrin monomers
|
Factor XIII
|
|
|
what would cause prolonged bleeding time
|
->thrombocytopenia - low platelet count (<90000) ->vWF disease - lack of vWF - most common genetic disease (autosomal dominant) ->taking an ASA - mechanism: block platelet cyclo-oxygenase
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|
|
platelet sequence
|
adhesion - release rxn (release ADP) - syn TXA2 - temporary plug
|
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|
test of platelet fxn to form temporary plug
|
bleeding time
|
|
|
what is the difference platelet disease and coagulation factor disease
|
with platelet disease - if scrap or scratch self have prolonged bleeding time because of cant from hemostatic plug -see epistaxis (nosebleed) - most common - petechia (pinpoint area of hemmorrhage in subcutaneous tissue) and eccymosis
-with coagulation disorders - have late (delayed) rebleeding after surgery or wisdom tooth extract bc lack of thrombin to convert fibrinogen to fibrin to stablize the platelet plug also see hematoma (from trauma) and/or hemarthroses (bleeding in joints) |
|
|
what questions can you ask pt to rule out coagulation deficiency
|
if pt bleeds from tooth extraction sites
|
|
|
ecchymoses - epitaxis - menorrhagia - hematuria - bleeding from tooth extraction sites - GI and intracranial bleeding
|
clinical features of coagulation disorders
|
|
|
PT or PTT evaluates factors XII - XI - IX - X - V - II - I
|
PTT
|
|
|
PT or PTT evaluates factors VII - X - V - II ( prothrobin) & I (fibrinogen)
|
PT
|
|
|
tests for vWF function
|
ristocetin cofactor assay
|
|
|
older pt with chronic headaches and osteoarthritis went in for surgery and is bleeding to death - check labs platelet count normal - PTT normal - how to tx this pt?
|
pt on NSAID for pain - platelets are non functional so tx with platelet pack transfussion to stop bleeding
|
|
|
Protime prolonged but PTT normal - what is the factor deficieny?
|
factor VII
|
|
|
PTT prolonged but PT normal
|
Factor VIII deficency
|
|
|
inhibits epoxide reductase and Vit K non fxn
|
warfarin
|
|
|
activiated in the liver by epoxide reductase
|
Vit K
|
|
|
dec in factor VIII : C amd VIII:Ag also inc PTT -> what disease?
|
Hemophilia A
|
|
|
warfarin prevents the gamma carboxylation of what factors?
|
Factors II & VII & IX & X
|
|
|
what test is used to monitor warfarin?
|
PT or INR bc more standardized - PTT is also dec but PT or INR measures it better
|
|
|
what coag factors are vit K dependent
|
F II & VII & IX & X & protien C and S
|
|
|
what is the best test for DIC (disseminated intravascular coagulation )
|
D - Dimer assay
|
|
|
blood test that only detects cross-linked insoluble fibrin monomers in a fibrin clot
|
D-Dimer assay
|
|
|
what test is used to screen for pulmonary thromboembolism why?
|
D-Dimer - bc it consists of rbc - platelets - wbc all held together by fibrin
|
|
|
most common cause of DIC
|
sepsis
|
|
|
consumption of coagulations factors
|
DIC - disseminated intravascular coagulation
|
|
|
normal age dependent finding seen in areas on the body that hit things
|
senile purpura on the back of hand - chin - any area normal for trama
|
|
|
most common genetic vascular disease characterized by red dots in skin - lips - tongue - and in GI tract
|
Osler-Weber-Rendu Syndrome also known as Hereditary Hemorrhagic Telangiectasia
|
|
|
associated with varfarin therapy in protien C deficiency
|
Hemorrhagic skin necrosis
|
|
|
pinpoint hemmorages 1. what is this 2. what is this a sign of 3. does this blanch with pressure
|
petechiae - platelet dysfxn - no
|
|
|
t or f spider angioma does not blanch
|
f it does bc arterial venous fistula
|
|
|
child with petechea on skin after upper respiratiory infection with a platelet count of 20000 (normal 150000 - 400000 cells/uL)
|
Acute idiopathic thrombocytopenic purpura (ITP)
|
|
|
most common thrombocytopenia in children
|
Acute idiopathic thrombocytopenic purpura (ITP)
|
|
|
the following is the moa for what type of thrombocytopenia? IgG Antibodies directed against GpIIb/IIIa receptors (hypersenitivity II) & macrophage phagocytosis of platelets
|
Acute idiopathic thrombocytopenic purpura (ITP)
|
|
|
type of thrombocytopenia -something in body damages small vessels through out body causing platelet aggregation - produces platelet plugs thoroug out all the small vessels in the body with following sx: -fever -renal failure -thrombocytopenia ** Must have schistocytes (damages rbc) in periperial blood with microangiopathic hemolytic anemia to make dx 1. in adults 2. in child
|
1. Thrombotic thrombocytopenic purpura (TTP) 2. Hemolytic uremic syndrome (HUS)
|
|
|
2 causes of Hemolytic uremic syndrome (HUS)
|
1. 0157:H7 serotype of E. Coli - organisms proliferate in uder cooked meat 2. shiga-like toxin primarly seen in children
|
|
|
what hemostasis disorder is the following? 1. low platelet count 2. inc bleeding time 3. normal PT and PTT
|
Thrombotic thrombocytopenic purpura (TTP) in adults and hemolytic uremic syndrome (HUS) in kids
|
|
|
what hemostasis disorder involves both platelet adhesion defect and coagulation factor defect
|
classic von Willebrand disease (vWD) autosomal Dominant with dec vWF and some dec in Factor VIII
|
|
|
hemostatic disease with following clinical presentation menorrhagia - epitaxis - easy bruisablity - association with agiodysplasia of right colon
|
classic von Willebrand Disease
|
|
|
x - linked hemostatic disease - only Factor VIII dec
|
Hemophila A
|
|
|
2 tx for vWD
|
1. Desmopressin (inc vWF and VIII: C) 2. oral contraception for women (estrogen act similarly)
|
|
|
what is the cause of Vit K deficiencey in hospitalized pt
|
due to antibiotic therapy
|
|
|
hemolytic disorder associated with presence of antiphospholipid antibodies (APA) directed against phopholipids bound to plasma protien and makde up of anticardiolipin Antibody and lupus anticoagulants what is the disease and other diseases associated with it
|
antiphospholipid synderome associated with SLE and HIV
|
|
|
what are common causes of DIC
|
sepsis due to ecoli (most common ) or neisseria meniginitis or rattlesnake bite
|
|
|
what hemostatic disorder presents with bleeding from every opening of the pt body?
|
DIC
|
|
|
most common cause of DIC
|
sepsis
|
|
|
prolonged PT and PTT - platelet count dec - D-Dimer positive -
|
DIC
|
|
|
best screen for DIC
|
D - Dimer assay
|
|
|
pt given heparin but PTT does not prolong - what is this condition?
|
Antithrombin III (ATIII) deficiency often seen in women on birthcontrol pills
|
|
|
young person presents with DVT and has a family hx of others with DVT
|
Factor V - Leiden
|
|
|
most common herdiatry thrombosis syndrome in which mutant form of factor V can not be degraded by protien C and S
|
Factor V Leiden
|
|
|
platelet count low - bleeding time prolonged - PT and PTT Normal
|
Acute Idiopathic thrombocytopenia purpura (ITP)
|
|
|
Platelet normal - bleeding time high - PTT & PT normal
|
ATIII deficiency
|
|
|
Platelet count - normal bleeding time - normal PT - normal PTT - prolonged
|
Hemophila A - Why bc deficiency in Factor VIII part of intrinsic pathway
|
|
|
platelet count - normal bleeding time - prolonged PT - normal PTT - prolonged
|
vWD bc dec in vWF and Factor VII
|
|
|
platelet count - normal bleeding time - normal PTT - prolonged PT - Prolonged Drug spefic??
|
PTT for Heparin PT (INR) for Warfarin
|
|
|
blood group type? anti - a - IgM and anti - b - IgM and most people have anti - ab - IgG antibodies
|
Type O
|
|
|
blood group type? Anti-B-IgM antibodies inc incidence of gastric carcinoma
|
A
|
|
|
Anti - A - IgM antibody
|
B
|
|
|
blood group type? least common with no natural antibodies
|
AB
|
|
|
antibodies found in newborn
|
IgG from mom
|
|
|
when do people frequently lose natural antibodies
|
the elderly
|
|
|
most common blood type
|
O
|
|
|
blood group associates with duodenal ulcers
|
O
|
|
|
blood type of new born
|
none bc newborn syn IgM when born
|
|
|
elderly pt with blood group A received blood group B by mistake and did not develop hemolytic transfution rxn
|
bc elderly lose natural antibodies as age
|
|
|
what blood type is a universal donor and why?
|
O - bc have no antigen on RBC membrane
|
|
|
what blood type is a universal receipant?
|
AB bc have no antibodies
|
|
|
Rh positive means what?
|
D antigen positive
|
|
|
binding site for infestation of rbc y plasmodium vivax - what population lact this antigen and what is the benefit of that
|
Duffy (Fy) antigen - protects black population from P. vivax malaria
|
|
|
major crossmatch
|
detects atypical antibodies that are directed against foregin antigents mix serum of pt with sample of donor rbc - look for lack of rbc agglutination or hmolysis indicating compatible ccross match
|
|
|
what is the down side to blood transfusions?
|
even if pt serum has minmal agglutination - no one person has the same antigens on rbc so pt will will develop antibodies to blood transfusion and each additional time there must be a antigens negative before transfusion
|
|
|
tests performed on donor blood
|
1. group (ABO) and type (Rh) 2. antibody screen ( indirect coomb's test) - detect atypical antibodies 3. screening for infectious diseases
|
|
|
most common pathogen transmitted by transfusion of blood
|
CMV
|
|
|
most common antibody in USA
|
antibody for CMV
|
|
|
what is used to tx multiple coagulation deficiencies such as DIC or Cirrhosis or for treatment of warfarin over - anticoagulation if bleeding is life treating
|
fresh frozen plasma
|
|
|
what reverses the over bleeding of heparin
|
protomine sulfate
|
|
|
how should a pt bp be inc
|
using saline not fresh frozen plasma
|
|
|
what is the most common trasfusion rxn
|
allergic rxn
|
|
|
what immunoglobulin is involved in allergic transfusion reaction?
|
IgE - mediated - type 1 hypersensitivity
|
|
|
what transfusion rxn has the following clinical characteristics? -urticaria with pruritus - fever - tachycardia - wheezing - potential for anaphylactic shock
|
allergic rxn with IgE mediated type 1 hypersenstivity
|
|
|
anti-HLA antibodies agains donor leukocytes
|
Febrile transfusion reaction
|
|
|
what transfusion rxn presents with fever - chills - headache - flusing ?
|
febrile transfusion rxn
|
|
|
what transfusion rx is a type 2 hypersensitivity?
|
febrile transfusion rxn
|
|
|
if a pt has never received any blood before does he or she have any anti-HLA antibodies?
|
No bc only develo anti-HLA antibodies when exposed to other human's blood
|
|
|
who is at most risk for developing febrile transfusion rxn
|
a woman that has been pregnant - with more pregnancies or spontaneous abortions has incr anti-HLA antibodies
|
|
|
transfusion rxn due to blood group incompatibility or presence of anatypical antibody
|
Acute Hemolytic transfustion reaction (acute HTR's)
|
|
|
group B pt receives group A donor blood what type of transfusion rxn?
|
acute intravascular hemolytic transfusion rxn
|
|
|
an atypical antibody rxt iwth a foreign antigen on donor RBC. macrophage phagocytosis and destruction of donor rbc coated by atypical antibody - jaundice commonly occurs
|
acute extravascular hemolysis transfusion rxn
|
|
|
what transfusion rxn presents with these clinical findings: fever - back pain - hypotension - disseminated intravascular coagulation - oliguria (renal failure)
|
acute hemolytic transfusion rxn
|
|
|
women post partum after difficult delivery. transfused 3 unites of blood. Hb = 10 when went home. one week later pt is jaundice and weak and Hb = 8
|
delayed hemolytic transfusion rxn
|
|
|
what test used when suspect acute or delayed hemlytic transfusion reaction
|
coombs test
|
|
|
most common cause of jaundice in newborn baby with in 24 hr
|
ABO incompatiblity
|
|
|
transplacental passage of maternal IgG antibodies (anti-D antibodies and anti-AB antibodies in O mothers)
|
extravascualr hemolytic anemia in fetus
|
|
|
what is used to treat newborn jaudice
|
UV-B (blue fluorescent) light because unconjugated bilirubin is converted to dipyrrole (lumirubin) which is water soluble and is urinated out
|
|
|
positive direct Coomb's test on fetal RBC
|
ABO hemolytic disease of newborn
|
|
|
which baby will have worse anemia? ABO incompatablilty or Rh incompatablilty
|
Rh
|
|
|
mom is O- and baby is A+ so is this a problem?
|
No - bc any fetal A+ RBC enters her circulation will be destroyed by materanl anti-A-IgM antibodies there by preventing sensitation
|
|
|
what prevents Rh HDN (hemolytic disease of newborn)
|
Rh immunoglobulin (anti-D globulin) - which pretects the mom from sensitation to fetal Rh + cells that may enter her circulation during the last trimester
|
|
|
unconjugated bilirubin deposits in basal ganglia bc of incomplete formation of bbb causing neuronal damage and sever dysfxn
|
kernicterus
|
|
|
what immunoglobulin is naturally occuring and is a fixed complement which may cause hemolysis?
|
IgM
|
|
|
blood group with amorphous allele where transferase adds no sugar
|
blood group O
|
|
|
80% of population have the ___________ that causes the presence of ABO substances in secretions ( saliva and tears GI and so on)
|
secretor gene
|
|
|
What antigens are most imp for clinical transfusion and solid transplancent
|
A B O antigen
|
|
|
what Rh group is very immunogenic and what antibodes are produces
|
RhD and IgG which do not occur naturally - from pregnancy or blood transfustion
|
|
|
what test is used to cross match the patient's serum with the donor's red cells and to detect the presence of IgG antibodyies in the patient's serum
|
indirect antiglobulin test (AGT) also called the indirect Coomb's test
|
|
|
What test is used to detecxt antibodies bound to rbc antigents in vivo
|
direct coomb's test also known as direct antiglobulin test
|
|
|
when should a blood type and screen be ordered?
|
if transfusion is possible but not certain (ie cholecystectomy and C section)
|
|
|
What transplantation reaction wold show the following clinical features? ->disseminated intravascular coagulation (DIC) ->acute tubular necrosis -renal failure
|
SEVERE HTR
|
|
|
The most severe consequences for hemolytic transfusion reaction?
|
renal failure and DIC
|
|
|
best way to prevent severe HTR?
|
Correct sample and patient identification
|
|
|
Caused by antibodies to WBC in the donor unit or by the presence of cytokines released by WBC
|
Febrile Reaction
|
|
|
Increased pyrogenic cytokines cause the hypothalamus to change the rate of thermoregulation What has this mechanism of action?
|
mechanism of febrile reaction
|
|
|
Caused by IgE antibodies to donor plasma proteins mostly IgA
|
allergic rnx of transfusion
|
|
|
Transfused allergen reacts with preformed IgE and activates mast cell
|
pathology of allergic reaction
|
|
|
Prevented by slow administration of blood 1ml/kg/hr ( normal 2ml/kg/hr for RBC) ; diuretics may be useful
|
circulatory overload
|
|
|
->Increase in plasma potassium - fresh blood for exchange transfusion ->Loss of 2 -3 DPG - increase in Hb affinity for oxygen - reversible in 24hrs
|
storage changes
|
|
|
what type of transfustion is contraincecated against infections and low Bed ?
|
autolytic transfusion
|
|
|
blood recovered and reinfused during surgery - clean only (excludes abdominal surgery and that for malignancy)
|
Salavge - a term used with autologous transfusion
|
|
|
what should not be given if the following occurs Should not be given if anemia can be corrected (iron and folate and B12 deficiencies)
|
salvage - autolougous
|
|
|
Documentation of the rationale for transfusion in the patient’s chart is very important Expected increment 1gHbdl for each unit transfused
|
RBC indication
|
|
|
Contraindicated in TTP and HUS and heparin
|
PLATELET TRANSFUSIONS
|
|
|
Transfused allergen reacts with preformed IgE and activates mast cells
|
PATHPHYSIOLOGY OF ALLERGIC REACTIONS
|
|
|
How is circulatory overload prevented?
|
slow administration of blood 1ml/kg/hr ( normal 2ml/kg/hr for RBC) ; diuretics may be useful
|
|
|
txmt Inc in plasma potassium
|
fresh blood for exchange transfustion
|
|
|
txmt loss of 2 -3 - DPG
|
inc in Hb affinity for oxygen - reversible in 24 hr
|
|
|
when is autologous transfusions contraindicated?
|
infections and low Hb
|
|
|
blood recovered and reinfused during surgery - clean only (excludes abdominal surgery and that for malignancy)
|
salvage
|
|
|
Should not be given if anemia can be corrected (iron and folate and B12 deficiencies)
|
rbc
|
|
|
when are platelet transfusions used prophylacticly?
|
?? To maintain a platelet count of 5-10000/microL ?? count above 50000/microL for surgery or invasive procedures
|
|
|
what contains all coagulation factors?
|
fresh frozen plama
|
|
|
Obtained by thawing FFP at cold temperature then stored frozen
|
CRYOPRECIPITATE
|
|
|
Good volume expander due to its sustained osmotic effect
|
ALBUMIN
|
|
|
transfusion of one blood volume (10 units) in 24 hrs
|
MASSIVE TRANSFUSION
|
|
|
How is coagulopathy measured
|
PT and APTT
|
|
|
maternal IgG antibodies to RBC antigen inherited from the father cross the placenta bind to fetal RBC and cause their removal by the reticuoendothelial system of the fetus
|
HEMOLYTIC DISEASE OF THE NEWBORN
|
|
|
A mother with what blood type with have a child affected with Hemolytic disease of Newborns
|
Type O
|
|
|
type of hemolytic disease of newborn (HDN) severe anemia and hepatosplenomegaly and increased unconjugated bilirubin - over 20mg/dl deposited in basal ganglia - kernicterus
|
moderate
|
|
|
what type of hemolytic disease of the newborn results in intrauterine death from hydrops fetalis
|
severe
|
|
|
how is Hemolytic anemia of newborns treated?
|
1. exchange transfusion - replace babies blood compatible with mother 2. phototherapy - degrade billirubin 3. simple transfusion to correct anemia
|
|
|
enoxaparin dalterparin tinzaparin
|
3 LMWH in US
|
|
|
factors necessary for heparin
|
ATIII and Factor Xa
|
|
|
factors for LMWH
|
need factor Xa
|
|
|
can only be partially reversed with protamine
|
LMWH
|
|
|
FDA approved drugs for HIT
|
lepirudin -> not available in us argatroban bivalirudin
|
|
|
direct thrombin inhibitor used for HIT in cancer tx but not FDA approved for management of HIT
|
dondaparinux
|
|
|
why are the indirect thrombin inhibitors have different half lifes?
|
bc each is elminated in a different way: argatroban - hepatic elmination bivalirudin - enzymatically eliminated (liver) Danaparoid - renal elimination
|
|
|
how are indirect thrombin inhibitors mostly monitored
|
aPTT
|
|
|
an adv of LMWH is dec osteoporsis - what population would benefit from this adv?
|
menopausal women - orthopedic surgical pt - pregnant women
|
|
|
Can the oral antithrombotic warfarin be used in HIT?
|
must waite to see - use when platelets > 50 1. use throbin inhibitor 2. platelet > 50 and better if 100 start warfarin at low doses
|
|
|
inhibits vit k dependent coag factors ( 2 - 7 - 9 - 10 and protien C and S)
|
warfarin
|
|
|
2 tests used for monitering warfarin
|
INR and Osmetech eSensor 2C9 / VKOR warfarin test for VKOR deficency and CYP2C9 polymorphisms. These 2 enzymes oxidize Vit K
|
|
|
irreversible inactivation of platelet cyclooxygenase lasting for the life of the platelet -> 5 to 7 days
|
asa
|
|
|
irreversible inactivation - ADP antiagonist - prevent platelet aggregation
|
clopidogrel (plavix)
|
|
|
interferes with adenosin uptake thereby reduicng platelet adhesion and aggregation - how long does this drug need to be dc before sugery
|
dipyridamole - 2 weeks
|
|
|
2 drugs that dissolve culprit vessel in a thrombus - inhancing plasminogen to plasmin - what indication can these drugs be used for?
|
streptokinase urokinase tPa - FDA approved to use in stroke within 3 hrs Indication: MI - pulmonary embolism - ischemic stroke
|
|
|
1. what is the AE of tPA
|
1. bleeding
|
|
|
what drug is used to tx hemophila A and B - what is it's AE
|
Factor 7 in extrinsic pathway - thromboembolisms
|
|
|
drugs used to tx gi bleeds but that also cause excessive thrombosis leading to DIC - bradycardia - urinary obstruction by promoting formation of bladder clots
|
E - aminocaprotic axid tranexamic acid
|
|
|
anti-hemophila drugs for hemophila A
|
factor 8 and vWF
|
|
|
anti-hemphilic products for hemophila B
|
factor 9
|
|
|
drug that controls hemorrhage inpt with mild to moderate hemophila A - willebrand diseases and uremic bleeding
|
desmopression - used for 24 hrs bc pt can develop tachyphylaxis in 48 hr
|
|
|
drugs used to tx uremic bleeding
|
conjugated estrogen
|
|
|
drug used for variceal and non-variceal gastrointestinal bleeding
|
somatostatin analogs (octreotide)
|
|
|
post-partum hemorrhage
|
vasopressin analog (oxytocin)
|
|
|
2 txmt for megoblastic anemia - how are they different
|
vit B 12 (cyanocobalamin) and hydroxycobalamin -hydroxycobalamin: longer acting
|
|
|
if pt has pernious anemia - why is giving folic acid cause pt to get worse?
|
folic acid will mask megablastic anemia - pt will get better histologically but neurologic symptoms will not improve and even may get worse
|
|
|
2 forms of tx for microcytic anemia pt
|
oral FE: tx for 4 - 6 mo parenteral FE: given to pt with acute losses that presist - given to pt with FE lost duing dialysis
|
|
|
1. what type of anemia does erythropoietin and darbepoetin tx? 2. What is the difference between the 2? 3. what is the se?
|
1. ACD 2. darbepotin has added glucose or sialic acid which prolongs the drugs half life which is great for outpt 3. inc thrombotic event and cancer activation
|
|
|
2 drugs used to manage transfusional iron overload shown to reduce iron induced complications such as diabetes and cardiac disease associated mortality
|
iron chelators: deferoxamine (parenteral) short half life and poor bioavalablity deferasirox (oral)
|
|
|
drugs used to tx CML
|
imitinib
|
|
|
tyrosine kinase inhibitor used to tx a type of leukemia
|
imitinab used to tx CML
|
|
|
2 agents used to circumvent BCR-ABL resistance
|
dasatinib and nilotinib approved for tx of imatinib-refractory CML
|
|
|
tyrosine kinase inhibitor known to cause peripheral edema
|
imatinib
|
|
|
tryrosine kinase inhibitor known to cause pleural edema
|
dasatinib
|
|
|
tyrosine kinase which CAN NOT be given with anti-secretory acid supplement therapy
|
nilotanib
|
|
|
fludarabine / cyclophosphamide (FC)
|
first line tx for CLL
|
|
|
Fludarabin / cyclophosphamide / rituximab (FCR)
|
first line tx for CLL
|
|
|
inhibit ribonucleotide reductase and inhibits DNA polymerase
|
fludarabin - part of CLL tx
|
|
|
inhibit rebonucleotide reductase and inhibit adenosin deaminase
|
pentostatin - 2nd line txmt for CLL
|
|
|
monoclonal antibody directed against CD-52 present on B and T lymphocytes
|
alemtuzumab - 2nd line txmt for CLL
|
|
|
drug that acts by inhibiting DNA methyltransferase which leads to hypomethylation of DNA - lead to apoptosis
|
azacytidine and decitabine - used to tx MDS
|
|
|
7day of anti-metabolite cytarbine and 3 days of anthracycline idarbucin (or daunorubicin) - what disease is this tx use for as first line tx?
|
AML -> t(15:17)
|
|
|
monoclonal antibody directed against CD 33 antigen which is expressed on leuckmic cells and immature myelomonocytic cells
|
gentuzumab - tx AML pt refractory cases
|
|
|
leukemia tx with all-trans retinoic acid
|
APL (AML3)
|
|
|
drug that binds to its native receptor RAR alpha to release the co-repressor complex and allows for normal maturaiton and differentiation of these cells
|
all-trans retinoic acid - tx AML3
|
|
|
platelet reducing agent used to reduce the maturation of megakaryocytes
|
anagrelide - used to treat myloproliferative disorders such as essential thrombocytopenia and polytemia vera
|
|
|
agents used to tx essential thrombocythermia
|
hydroxyurea - anagrelide - interferon alfa - 2a
|
|
|
management for polycyethemia vera
|
low dose asa and reverse thrombotic risk factors
|
|
|
management of patients with essential thrombocythemia (ET)
|
reverse cardiovascular risk and with pt over 60 and platelet > 1500 x 109 - low dose asa plus hydroxyurea
|
|
|
CVP - cyclophosphamide / vincristine / prednisone
|
traditional low - grade lymphoma tx
|
|
|
monoclonal antibody against CD 20 on B cells activates antibody dependent cytoxicity - can lead to cytokine release manifested as infustion related rxn
|
rituximab - used to tx non-hodgkins lymphoma
|
|
|
2 drugs used to tx refractory cases of NHL
|
ibritumomab Tiuxetan and tositumomab
|
|
|
mainstay txmt of diffuse large B-cell lymphoma
|
CHOP -
|
|
|
lymphoma tx by hyper-CVAD regimen -cyclophosphamide -vincristine -doxorubicin -dexamethadose -methotrexate -cytarabine
|
mantle cell lymphoma
|
|
|
txmt regimen used to tx HD but causes more sterlity - which of the drugs are alkalyting agents?
|
MOPP - mechlorethamine / vincrestine / prednisone / procarbazine - alkalyating agents are vincrestine and procarbazine
|
|
|
stardard of care for tx of HD esp in young pt - which of the drugs in the regiment are alkalyating agents
|
ABVD - adriamycin / bleomycin / vinblastine / dacarbazine -alkalyating agents: adrimycin and vinblastine
|
|
|
goals of chemotherapy
|
1. create space in bm for donated cells 2. suppression of pt immune system to prevent rejection 3. eradicate present tumor
|
|
|
chemotherapeutic agent which causes cardiomyopathy at very high doses
|
cyclophosphamide
|
|
|
drug used in pt who would otherwise not be eligible for conventional alllogeneic stem cell transplantation
|
fludarbine - purine analogue
|
|
|
what cells involved in ALL and what markers are involved?
|
Pre -Pre B-cells and Pre-Cells Markers: CD10 (CALLA) and TdT (terminal deoxynucleotidyl transferase
|
|
|
markers for: 1. early B-cells 2. mature B-cells
|
1. TdT 2. surface Immunoglobulin (sIg)
|
|
|
>1 cm lymph node; exception: inguinal ares (>2 cm)
|
lymphandemopathy
|
|
|
>3 non-contiguous area of enlargement
|
generlized lymphadenopathy
|
|
|
benign or malignant lymph node hyperplasia
|
benign
|
|
|
benign or malignant leukemia and lymphoma
|
malignant
|
|
|
total lymphocyte count is normal but percentage increased - most condition associated with neutropenia
|
relative lymphocytosis
|
|
|
With what virus infection is it very common causes lysis of infected lymphocytes ?
|
HIV
|
|
|
bizare morphology with convoluted "clover-leaf" nucleus
|
Adult T-cell leukemia / lymphoma (ATLL)
|
|
|
what lymphoma is being described: 1. characteristic phenotype: CD5+ 2. characteristic karyotype: t(11:14) 3. overexpression of Cylin D1/BCL1 4. incurable - fast growing - aggressive
|
mantle cell lymphoma
|
|
|
what leukemia and lymphoma both have the characteristic phenotype of CD5+
|
Mantle Cell Lymphoma and Chronic Mylogenic Leukemia (CML)
|
|
|
What 2 chronic lymphoid leukemias have Massive splenomegaly without lymphadenopathy
|
Prolymphocytic Leukemia (PLL) and Hariy Cell Leukemia (HCL)
|
|
|
oval nucl - finaly mottled pal - gray/blue cytoplasm with irregular edge - pancytopenia - shaggy cytoplasm edge
|
Hairy Cell Leukemia (HCL)
|
|
|
lymphadenopathy - hepatosplenomegaly - skin lesions - CD4+ - related to bone reabsorption and bone pain
|
ATLL = Adult T-cell Leukemia / Lymphoma
|
|
|
3 signs of BM failure
|
1. anemia 2. thromocytopenia 3. neutropenia
|
|
|
what stage of CLL? absolute lymphocytosis > 15 x 109/lm 40% lymphocytes in BM txmt?
|
Stage 0 txmt = observe
|
|
|
what stage of CLL? stage 0 + enlarged lymph nodes (adenopathy) txmt?
|
Stage I txmt = tx selected cases if enlarged lymph nodes and spleed or liver with cytotoxic agents - corticosteroids or monoclonal antibodies
|
|
|
what stage of CLL? stage 0 + enlarged liver and/or spleen + andenopathy txmt?
|
Stage II txmt = tx selected cases if enlarged lymph nodes and spleed or liver with cytotoxic agents - corticosteroids or monoclonal antibodies
|
|
|
What CLL stage? stage 0 + anemia (Hb < 11.0 g/dl) + adenopathy + organomegaly
|
Stage III - tx with cytotoxic agents - corticosteroids or monoclonal antibodies
|
|
|
What CLL stage? stage 0 + thrombocytopenia (platelet < 100x 109 / l) + adenopathy + organomegaly
|
Stage 4 - tx with cytotoxic agents - corticosteroids or monoclonal antibodies
|
|
|
A complication of CLL is transformation - what transformation takes place?
|
CLL is transformed to Diffuse Large B-Cell Lymphoma (DLCL) also referred to as Richter's Syndrome or prolymphocytic leukemia (PLL)
|
|
|
what is Richter's syndrome
|
another name for diffuse large cell lymphoma (DLCL) the transformation from CLL
|
|
|
bone marrow "dry tap" secondary to fibrosis and cells staing positive for tartrate - resistant acid phosphatase (TRAP)
|
hairy cell leukemia
|
|
|
what Ann Arbor stage for HL? 1 area of nodal involvement
|
stage I - early disease
|
|
|
what Ann Arbor stage for HL? 2 or more areas of nodal involvement above the diaphragm
|
Stage II
|
|
|
what Ann Arbor stage for HL? multiple areas of nodal involvement above and below the diaphargm
|
Stage III
|
|
|
what Ann Arbor stage for HL? bone marrow infiltrated
|
Stage 4
|
|
|
What is the exception to Stage 4 Ann Arbor for HL
|
when extranodal spread from lymph node to bone marrow referred to as Stage IE
|
|
|
what 2 lab values are a MUST for HL?
|
1. bone marrow aspirate and trephine 2. LDH - marker for HL -> also marker for rapid cell turner over -> Dx
|
|
|
what lymphoma is senstive to both radation and chemotherapy?
|
HL
|
|
|
what disease has the following hemotolgy and chemistry? 1. anemia - neutropenia - thrombopenia 2. circulating lymphoma cells in blood 3. Lactate Dehydrogenase (LDH) - elevated in aggressive lymphoma indicates poor prognosis 4. tumor lysis syndrome (B-cells release content) - in high grade lymphoma - hyperkalemia - hyperphosphatemia - hyperuricema - hypocalcemia
|
NHL
|
|
|
What disease? t(14;18) & bcl2
|
follicular Lymphoma
|
|
|
What disease? t(11;14) & bcl1
|
Mantle cell Lymphoma (MCL)
|
|
|
What disease? t(8;14) & c-myc
|
burkitt's lymphoma
|
|
|
presents with pruritic - psoriasis - like plaque lessions and involving chronic cutaneous T - cell lymphoma usually CD4+ also see Sezary syndrome
|
Mycosis Fungosides (MF)
|
|
|
MF and circulating lymphoma cells (folded and cereriform nuclei)
|
Sezary Syndrome
|
|
|
what disease? 1. eccentric nuclei with deep blue cytoplasm with perinuclear halo 2. lytic bone lysions due to bone resorption 3. monoclonal gammopathy (expansion of one clone of Ig-secreting cell on serum protien electrophoresis)
|
Multiple Myeloma (MM)
|
|
|
clinical features of what disease? --bone pain -renal failure - multifactoral -hypercalcemia secondary to bone resorption -amyloidosis - light chain deposits -hyperviscosity syndrome bc of inc Ig
|
Multiple Myloma
|
|
|
what is this ->disorder characterized by extracellular deposition of protien in abnormal fibrillar form what diseases have this?
|
Amyloidosis seen in: - Multiple Myeloma (MM) - Waldenstrome's Myacroglobulinemia (WM) - Monoclonal Gammopathy of Undetermined significance (MGUS)
|
|
|
M-protien levels (IgM) < 3 gm/dL <10% Plasma Cells in BM Normal serum Calcium - renal fxn No anemia and no bone lesions
|
Monoclonal Gammopathy of Undetermined significance (MGUS)
|
|
|
what disease? seen in older pt (average = 64) with fatigue/bleeding and weight loss hepatosplenomegaly and lymphoadenopathy lymphoplasmacytoid Always IgM Hyperviscosity is common
|
Waldenstrom's Macroglobulinemia
|
|
|
-immature red blood cells - typically composing about 1% of the red cells in the human body -circulate for about a day in the blood stream before maturing -mesh-like network of ribosomal RNA that becomes visible under a microscope with certain stains such as new methylene blue
|
Reticulocytes
|
|
|
what types of anemia have low reticulocyte count
|
macrocytic anemia due to vit B12 deficiency and Folate deficiency
|
|
|
What type of anemia is due to high reticulocyte count?
|
microcytic anemia - inc from 1 - 2%(normal) to 4 - 6% and as high or greater than 15%
|
|
|
what type of anemia? -intramedullary hemolysis -MCV = 120 -dec Hgb and wbc and platelet -very low reticulocyte count -very high iron in marrow
|
macrocytic anemia - vitamin deficiency
|
|
|
what makes reticulocytes to mature and how long does it take for them to mature?
|
erthropoietin and it takes 4 days
|
|
|
disease with ince basophils and inc platelets and inc precursors of bm
|
cml
|
|
|
disease where platelets in peripherial blood is > 600 k/ul
|
essential thrombocytosis
|
|
|
what phase of cml is characterized by inc in reticulin fibrosis and no aspirate "dry tap"
|
spent phase of cml
|
|
|
acquired defect at stem cell level produce sever structural abnormalites during maturation with premature cell destuction
|
myelodysplastic syndromes
|
|
|
what disease? pt has peripheral cytopenia - hypercellular bm - cytologically abnormal cells in bm and peripheral blood
|
myelodysplastic syndromes
|
|
|
what are pesudo pelger huet cells and when would you see these cells
|
pmn with only 1 or 2 lobes - seen in myelodysplatic syndromes
|
|
|
blasts must be >20% of the cellularity for a diagnosis of ____________
|
acute leukemia
|
|
|
Blast count of <20% are seen in are seen in what 2 syndromes
|
myeloproliferative and myelodysplastic syndromes.
|
|
|
type of leukemia consists of blasts of either T cells or B cells
|
ALL
|
|
|
type of leukemia consists of blasts with characteristics of myeloid cells (granulocytes &monocyte & megakaryocytes & erythrocytes)
|
AML
|
|
|
what is the reason for iron loss in adults vs children
|
adult - blood loss child - dietary insuffiency
|
|
|
3 cell type that have ferroportin receptors
|
mucosal cells / reticuloendothelial cells and hepatocyte - bind transferrin with Fe
|
|
|
water soluble iron-protein complex made up of a protein shell and an iron-phosphatehydroxide core. Iron constitutes up to 20% of the weight of the molecule.
|
Ferritin
|
|
|
water insoluble storage iron complexes that results from the partial digestion of ferritin. They represent another form of storage iron. Granules of thes substance seen in interstial area near macrophage
|
Hemosiderins
|
|
|
state of iron overload where iron has saturated various cells in body such as endrocrine glands - pancrease - heart - hepatocyte causing damage to the organs
|
hemochromatosis
|
|
|
Controls movement of iron. Protien made in the hepatocyte. Inhibits iron absorption by dampening the ferroportin.
|
Hepcidin
|
|
|
Hemochromatosis gene and resultant controller substance for the transferrin receptor
|
HFE
|
|
|
receptor which provides attachment of molecules such as iron and zinc on the luminal end of mucosal cells in duodenum and jejunum allowing entry of these molecules into the cell
|
DMT - 1 = divalent metal ion transfer protien
|
|
|
what protien is found in the base of cells in the crypt of microvilli in the duodenum and jejunum and provide attachment of transferrin and iron uptake
|
HFE = hemochromatosis protien
|
|
|
what effect does an inc in ferritin have on divalent metal transporters?
|
dec activity of transporter
|
|
|
• Microcytic anemia • May be hypochromic • Low serum ferritin • Low serum iron and a saturation of the TIBC less than 10%. • Blood film shows MCHC red cells and anisicytosis. what type of anemia is this?
|
iron deficiency anemia
|
|
|
what anemia has the following mechanism? • Shortened RBC survival (40-80 days). • Failure to increase the erythropoitin in response to the anemia. ->The anemia is limited - most often no lower than 8.0 gm/dl. ->The serum ferritin is normal or increased also iron is low but the saturation of the TIBC is 10- 25%. ->NORMOCYTIC (ACCORDING TO LECTURE)
|
ACD
|
|
|
what type of anemia has the following causes: • Inherited as an X linked recessive disorder. • Acquired due to: – A myelodysplastic disorder (RARS) – Secondary to malignant diseases. – Due to drugs (chloramphenacol) or toxins (alcohol or lead)
|
sideroblastic anemia
|
|
|
what anemia has the following characteristics? The common feature is that there is a failure to form heme though the building blocks are available. If there is iron available but protoporphyrin is not and the iron accumulates in the mitochondria. If iron cannot be plugged into the porphyrin then the iron accumulates.
|
sideroblastic anemia
|
|
|
any state of iron overload with no organ damage
|
Hemosiderosis
|
|
|
what diseases can secondarly lead to hemochromatosis?
|
thalassemia PK deficiency other hemolytic states
|
|
|
|
|
