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56 Cards in this Set
- Front
- Back
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A. general rule of microcytic vs macrocytic
B. MCV C. MCH D. MCHC E. RDW |
A. microcytic: disorders of hemoglobin syn
- macrocytic: impaired maturation of erythroid precursor B. ave volume of red cell C. ave mass of Hb per red cell D. ave concen of Hb in given RC volume E. coeff of variation of red cell volume |
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Anemia of Blood loss
A. acute B. chronic |
A. loss of intravascular volume --> compensatory release of adrenergic hormones --> leukocytosis, reticulocytosis, thrombocytosis
- note: reticulocyosis: larger tan RBC + red/blue cytoplasm |
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Hemolytic anemia
A. name the common features |
- prmature RBC destruction (shorted life span) = anemia
- erythropoietin incr - accumulation of degradation products = jaundice |
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Hemolytic anemia
A. name them |
- red cell membrane: hereditary spherocytosis
- enzyme def: G6PD deifciency, pyruvate kinase - hemoglobin abnl: thalassemia, sickle cell - acquired genetic dfect: paroxysmal nocturna hemoglobinuria - mechanica: Microangiopathic (HUS, TTP), defective cardiac valves - sequestration |
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A. extravascular hemolysis
B. intravascular hemolysis C. hallmark of chronic hemolysis |
A. occur within phagocytes = in spleen
- anemia, splenomegaly and jaundice - decr haptoglobin b/c bind to Hb B. anemia, hemoglobinemia, hemoglobinuria, hemosdiernuria, jaundice C. elevated biliary excretion = cholelithiasis |
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Hereditary spherocytosis
A. pathogenesis |
- insuf of membrane skeletal components = instablity
- yong cells normal but loss of membrane in circ = spheres - erythrostasis (prolonged splenic exposure) - phagoctosis, exravascular hemolysis |
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Hereditory spherocytosis
A. smear hallmarks B. dx C. consequences |
A. hyperchromic RBC w/o central pallor
B. most enlarged spleen - sensitive to osmotic lysis - incr MCHC b/c dehydration by loss of K and H20 in erythrostasis C. aplastic crisis: trigered by acute parvovirus - hemolytic crisis: incr splenic destruction due to mono |
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Glucose-5-Phosphate dehydrogenase decifiency
A. role of G6PD B. genetics |
A. reduces NADP TO NADPH which reduces glutathione to protect against oxidant injury
B. x-linked |
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G6PD deficiency
A. hallmarks B. special note |
- intravascular hemolysis: oxidized globin chains form membrane-bound precipitates Heinz bodies that damage membrane
- Extravascular: bite cells = spleen macrophages take out Heinz bodies + spherocytes also B. occurs in older cells so intermittent = no chronic hemolysis = no cholelithiasis or splenomegaly |
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Sickle Cell Disease
A. molecular patho B. relationship to transit time C. mechanism of RBC change |
A. glutamate substituted w/ valine = HbS polymerize when deoxy = cytosol cover to viscous gel
B. slow when sickling and in INFLAMED vascular beds - b/c sickle cells have > ahesion molecules - leukocyte count correlates w/ freq of pain crises C. as HbS polymer grow --> herniate membrane skeleton = efflux of K, H2O = dehydration |
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Sickle cell
- morphology |
- sickled cells, reticulocytosis, target cells (from dehydra)
-Howell Jolly bodies (nuclear remnants) form asplenia - crew-cut x-rays b/c bone resorption and sec new bone formation - extramedullary hematopoiesis --> incr brkdown = gallstone |
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Sickle Cell
- sx |
- autosplenectomy: chronic erythrostasis --> fibrosis, infaction
- vaso-occulsive crisis: acid, dehy, infxn promote sickle - chest syndrome: from pulmonary inflammation - sequestration crses: entrap sickle cells - incr susceptibiliyt to encap organisms |
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sickle cell
A. tx |
- hydroxyurea: inhibitor of DNA synthesis
- above increases HgF and anti-inflammatory |
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beta-Thalassemia
A. molecular pathogenesis |
A. impaired beta chain syn --> unerhemoglobines hypochromatic, microcytic RBC
- unpaired alpha chains precipitate --> inclusions --> membrane damage --> sequestration + extravascular hemolysis - ineffective erythropoiesis in severe form --> hyperplasia in marrow, extensive extramedulary hematopoiesis --> excessive absorption of dietary iron |
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Beta-thalaasemia major
A. dx |
- low hemoglobin leve
- major hHb is HbF (elevated) - bony promineses enlarged = extramed hematopoeisis - hepatosplenomegaly from extramed hematopoesis |
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Beta-thalassemia
A. morphology |
- anisocytosis (size) and poikilcytosis (shape)
- targe cells (Hb in center of cell), basophilic stippling - reticulocyte count elevated but low for expected anemia - hemosiderosis and seconary hemachromatosis |
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alpha thalassemia
A. vs beta B. types |
A. hemolysis and ineff erthryopoiesis less severe b/c beta & gamma chains more soluble
B. silent carrier state: one deletion of globin gene - alpha-thalassemia trait: deletion of two genes = no anemia, microcytosis - Hemoglobin H dz: del 3 genes --> tetramers of beta globin = HbH with high O2 affinity + > oxidation = sequestration - Hydrops fetalis: del all 4 --> gamma chains form tetramers Hb barts = high O2 affinity. |
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Paroxysmal nocturnal hemoglobinuria
A. def B. molecular patho |
A. acquired genetic defect of PIGA = enzyme for syn of surface proteins
B. mut in stem cell --> affect red/white/platelets - appear when it has selective adv ie autoimmune dz - CD59 neg - no inhibitor of C3 convertase = intravas hemolysis by complement esp at night when pH lower - thrombosis leading cause of death (no platelets) |
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Immunohemolytic anemia
A. direct coombs B. indirect Coombs C. classification of types |
A. patient's RBC mixed with Ig
B. patient's serum tested to agglutinate RBC A. warm antibody type: IgG coat RBC bind Fc phagocytes -- partial phagocytosis --> spherocytes from membrane loss - cold agglutinin: IgM bind RBC at low temp in mycoplasma, EBV, CMV, HIV. binds in periphery and fixes complement rapidly in warm areas - cold hemolysin: IgG bind to P blood group antigen in peripheral and complement lysis when cells recirc to warm central areas |
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Anemia of diminished erythropoiesis
A. name |
- megaloblastic anemia (B12/folate acid def)
- iron deficiency anemia - aplastic anemia - pure red cell aplasia |
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Megaloblastic anemia
A. etiology and reason B. morphology |
A. B12 and folic acid def needed for thymidine syn
B. MCHC not elevated - retic count low - neutrophils hyperseg - megakaryocytes multilobuated - derangement of DNA in marrow __. pancytopneia |
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Vit B12 def
A. normal metabolism B. fxn of B12 |
A. salivary gland releases R-binder that binds to pepsin released B12 in the stomach
- pancreatic proteases split the complex -> IF (by parietal cells of fundic mucosa) binds to B12 --> abs in ileum B. homocysteine to methionine = gen FH4 that converts dUMP --> dTMP - 2nd fxn: methylmalonyl to succiny. if not, abnl fatty acid accum in neuron = myelin breakdown |
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Vit B12 def
A. tx caveat B. pathogenesis |
A. folate acid releaves anemia but not address neurologic sx of B12 def
B. chronic atrophic gastritis - autoantibodies |
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Vit B12 def
A. morphology B. long term effect |
A. atrophy of fundic glands
- intestinalization: glandular lining in stomach replaced by mucus secreting globlet cells - atrophic glossitis (shiny, beefy tongue) - CNS lesions: demyelination of dorsal/lateral tracts B. risk of gastric carcinoma |
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Vit B12
A. dx criteria |
- magloblastic anemia
- luekopenia w/ hyperseg granulocytes - low serum vit B12 - incr homocystein & methylmalonic acid |
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Folate deficiency
A. biological importance B. etiology |
A. make FH4 = one-carbon swaps & make dTMP
B. decr intake, incr requirements, impaired utilization |
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Iron Deficiency anemia
A. role of hemosiderin and ferritin B. how is iron transported |
- free iron sequesterd by ferritin or hemosiderin
- hemosiderin = complex of ferritin * stains blue in Prussian blue stain - in nl iron stores, trace hemosiderin - ferritin correlate w/body iron stores B. transferrin made by liver |
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Iron def
A. iron regluation |
- regulated by proximal dueodenum
- hepcidin made in liver inhibits Fe transfer by binding to ferriportin (endocytosed, degraded) - Fe trapped within duodenal cells and lost when cells sloughed off - hepcidin also sup Fe release from macrophages (trouble in anemia of chronic dz) |
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Iron def
A. clinical rule B. lab findings C. morphology |
A. iron def is resultant of gastrointestinal blood loss until proven
B. low serum iron, ferritin + incr Fe binding capacity = transferrin saturation C. disappearance of stain on Prussian blue stain - microcytic, hypochromic, palor enlarged |
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Iron def
A. sx |
- pica
- Plummer-Vinson syndrome: esophageal wbes, atrophic glossitis, anemia |
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Anemia of chronic dz
A. lab findings B. assoc dz |
A. incr storage of Fe in marrow macrophages
- low serum ferritin level, reduced iron-bindng capacity - hepcidin incr B. microbial infxn (osteomyelitis, endocarditis), immune (RA), neoplasms (Hodgkin) |
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Aplastic Anemia
A. def B. morphology C. hallmarks |
A. chronic primary hematopoietic failure + pancytoplenia
B. hypocellular bone marrow with only fat cells - dry tap in aspirates --> get biopsies - abld bleeding + easy infxn C. splenomegaly absent, reticulocytopenia |
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Pure red cell aplasia
A. etiology |
- autoimmune basis --> plasmapheresis or AI therapy
- thymoma - parvovirus B19: destrys red cell progenitors |
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Bone marrow failure
- name additional types |
- myelophthisic anemia: space-ocupyig lesions replace marrow
- see teardrop shaped red cells b/c deformed during tortuous escape from fibrotic marrow - chronic renal failure (no erythropoietin) hepatocelluar liver dz |
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Hemorrahgi diathese
A. etiology B. prothrombin time C. partial thromboplastin time |
A. incr fragility of vessels
- platelet def or dysfdn - derangement of coagulation B. extreinsic pathway (def fact 5, 7, 10, prothrombin) C. intrinsic def (interfering Ig to phospholipid, above) |
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Nonthrombocytopenic purpura
A. def B. lab findings C. etiology |
A. caused by vessel wall abnormalities
B. nl platelet ct, bleeding time, PT, PTT C. infxn (meningococcemia, rickettsioses) - drug rxn - scurvy, Ehlers-Danlos syn - Henoch-Scholnlein purpura: rash, abd pain, polyarthralgia - hemorrhagic telangiectasia: most bleeding - Cushing synd: protein-wasting effects of excess corticosteriod prod |
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Bleeding: thrombocytopenia
A. threshold for overt sx B. etiology |
A. below 20,000
B. decr platelet prod (HIV) - decr platelet survival (autoimmune, DIC, microagniopath) - sequestration (when spleen enlarged) - dilusion (transufsion) |
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Bleeding: thrombocytopenia
A. name the special types |
- chronic immune thrombocytopenic purpura (ITP)
- acute ITP - drug-induced thrombocytopenia - HIV-assoc thrmbocytopenia - thrombotic thrombocytopnic purpura (TTP) - Hemolytic-uremic syndrome (HUS) |
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Chronic immune thrombocytopenic purpura
A. pathogenesis B. morphology |
A. IgG antiplatelet = opsonins --> bind Fc receptor of phagocytes --> removed in spleen (tx splenectomy)
B. spleen nl size but congesting of sinusoids - marrow: incr & megakaryocytes - peripheral blood: abnl large platelets (megathrombocytes) = sign of accel thrombopoiesis |
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Chronic ITP
A. clinicla sx B. lab findings C. tx |
A. petechiae (pinpoint hemorrhages)
- hx of bruising, nosebleeds, bleeding from gums B. low platelt ct, incr megakryocytes, large plagelets - PT, PTT nl C. glucocorticoids, anti-CD20 Ig (rituximab) |
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Acute immune thrombocytopenic purpura
A. epidemio B. sx |
A. childhood
B. following viral illness, self-limitd |
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Drug Induced thrombocytopenia
A. examples of drugs B. Haparin-induced thrombocytopenia: types |
A. vancomycin, quinine, quinidine
B. type I thrombocytopenia: no sig type II: venous/arterial thrombosis due to binding of Ig to platelet --> thrombosis even if thrombocytopneic |
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HIV-assoc thrombocytopenia
A. pathogenesis |
A. megakaryocytes has CD4 * CXCR4 --> cells infected --> prone to apoptosis and not produce platelets
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Thrombotic thrombocytopneic purpura (TTP)
A. hallmark sx B. molecular pathogenesis |
A. fever, thrombocytopenia, renal failure
- microangiopathic hemolytic anemia - transient neurologic dificits B. assoc with ADAMTS13 --> degrade multimers of vWF |
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Hemolytic-Uremic Syndrome (HUS)
A. sx B. typical type C. atypical type |
A. microangiopathic hemolytic anemia, thrombocytopenia
- acute renal fiailure, in children - no neurologic sx, nl ADAMTS13 B. infx gastroenteritis by E.coli O157:H7 (shiga-like toxin) - defect proteins that nlly prent excessive activation of alternative complement pathway |
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differentiate HUS/TTP form DIC
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TTP & HUS: nl PT, PTT
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Bleeding disorder: defective platelet fxn
A. etiology B. acquired causes |
A. defect in adhesion (Bernard-Soulier syn: glycoprotein Ib)
- deft of aggregation (Glanzmann thrombasthenia: IIb-IIIa) - defect of platelet secretion: storage pool disorders B. aspirin: irrev inhibit ccloxygenase = no syn thromboxane A2 + prostaglandins |
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Abnl in Clotting factors
A. sx B. hereidatry def vs acquired |
A. no petechiae, large post-traumatic ecchymoses
B. hereditary: affect single clotting facor - acquired: multiple coagulation |
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Factor VIII-vWF complex
- explain physiological fxn |
- VIII - cofacto of IX that converts X to Xa
- VIII made in liver/kidney and vWF in endothelial cells/megakaryocytes - form complex in circumlaion = longer half-life of VIII - vWF also in subendothelial matrix of blood vessels - vessel damaged --> vWF exposed --> help adhesion of platelets - circ vWF complex further hlep platelet adhesion |
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Von Willebrand dz
A. dx test B. lab findings |
A. ristocetin agglutination test
B. no vWF --> decr VIII in plasma --> PTT prolongation |
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Hemophilia A
A. def, egentics B. sx C. lab findings D. explain C |
A. x-linked factor VIII def
B. no petechiae, but hemarthroses and massive hemorrhage after trauma C. prolonged PTT, nl PT D. role of extrinsic pathway = initiate thrombin activation. thrombin then activates XI for intrinsic |
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Christmas disease
A. def B. sx |
A. Factor IX def
B. sx indistinguishable from factor VIII def |
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Disseminated intravascular coagulation (DIC)
A. def B. etiology |
A. excessive coagulation activation hat leads to thrombi formation in microvasculature
B. trigerred by release of tisue factor or injury to endothelial cells - clotting then initated by extrinsic (release of tissue factor (tissue thromboplastin) or intrinsic (activation of XII by surface contact w/ collagen) |
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DIC
A. etiology of endothelial injury B. consequences |
A. obstetric complications, neoplasms (adenocarcimnoma of lung) sepsis
B. widespread deposition of fibrin --> ischemia - microangiopathic hmoelytic anemia b/c RBC squeeze - consumption --> reduce clotting factor concen |
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DIC
A. normally how is coagulation prevented |
- imp for clotting to be limited to site of injury
- when thrombin swept away, converted to anticoagulant by binding to htrombomodulin - thrombin-thrombomodulin complex activates rotein C that inhibits factor V and VIII (other factors removed by liver) |
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DIC
- organ morphology |
- kidneys: thrombi in glomeruli--> reactive swelling of endothelial cells
- lungs: hyaline membrane due to thrombi in alveolar capillaries - adrenal: Waterhouse-Friderichsen syn (after meningococcemia) - obstetrics: sheehan postpartum pituitary necrosis |
