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250 Cards in this Set
- Front
- Back
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Erythropoietin
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synthesized in peritubular capillaries
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Reticulocyte count
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measure of effective erythropoiesis; correct for degree of anemia
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Extramedullary hematopoiesis
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hematopoiesis outside bone marrow (e.g., spleen)
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Newborn physiologic anemia
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drop in Hb due to replacement of HbF RBCs with HbA
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Pregnancy Heme
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Hb and Hct decreased; greater increase in plasma volume than RBC mass
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Anemia
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normal 02 saturation and arterial P02
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MCV
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average volume of RBCs; useful for anemia classification
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MCHC definition
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average Hb concentration in RBCs
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MCHC specifics
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dec in microcytic anemias; inc in spherocytosis
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Thalassemias
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dec MCV, inc RBC count
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RDW
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RBC size variation; inc iron deficiency; normal in other microcytic anemias
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Mature RBC
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anaerobic glycolysis; no mitochondria or HLA antigens
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Total iron binding capacity
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inc iron deficiency; dec anemia chronic disease, sideroblastic anemia
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% Saturation
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dec iron deficiency, anemia chronic disease; inc sideroblastic anemia
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Serum ferritin
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dec in iron deficiency; inc anemia chronic disease, sideroblastic anemia, normal thalassemia
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Microcytic anemias
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iron deficiency MC, anemic chronic disease, thalassemia, sideroblastic anemia
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Iron deficiency child
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MCC Meckel's diverticulum
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Iron deficiency woman < 50
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MCC menorrhagia
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Iron deficiency man < 50
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MCC peptic ulcer disease
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Iron deficiency men/woman > 50
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MCC colon cancer
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Stages iron deficiency
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dec ferritin; de Fe and % saturation, inc TIBC; normocytic then microcytic anemia
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Anemia chronic disease
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MC anemia in malignancy and alcoholics
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a-Thalassemia trait
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AR; two a-globin gene deletions; normal Hb electrophoresis
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HbH disease
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three a-globin gene deletions; hemolytic anemia; four beta-globin chains
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Hb Bart's disease
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four a-globin gene deletions; four gamma-globin chains
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beta-Thalassemia minor
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AR; DNA splicing defect; T HbA2 and F; .L HbA
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beta-Thalassemia major
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nonsense mutation with stop codon; hemolytic anemia; TTHbF, T HbA2
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Sideroblastic anemia
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defect in mitochondrial heme synthesis producing ringed sideroblasts
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Causes sideroblastic anemia
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alcohol, pyridoxine deficiency (isoniazid Rx of TB), Pb poisoning
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Pb poisoning
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inhibition ferrochelatase, S-aminolevulinic acid dehydrase, ribonuclease
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S/S Pb poisoning in children
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growth retardation; Pb in epiphyses; abdominal colic; encephalopathy
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S/S Pb poisoning in adult
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peripheral neuropathy; proximal renal tubule damage (Fanconi's syndrome)
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Labs in Pb poisoning
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coarse basophilic stippling RBCs; dec MCV; inc blood Pb; inc S-aminolevulinic acid
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Vitamin B12:
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animal products; requires intrinsic factor for reabsorption in terminal ileum
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Vitamin B12 biochemical function
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transfers methyl group to homocysteine
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R factor
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binds with B12 in mouth, removed by pancreatic enzymes in small intestine
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Vitamin B12 involvement in metabolism
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involved in propionate metabolism; end-product succinyl CoA
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Causes of B12 deficiency
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vegan, pernisicious anemia MC, fish tapeworm, pancreatitis, bacterial overgrowth, Crohn's disease
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Pernicious Anemia
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autoimmune destruction of parietal cells; chronic gastritis body/fundus; achlorhydria; inc gastrin
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Causes of folate deficiency
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alcohol MCC, poor diet, drugs, malabsorption, pregnancy, goat milk
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Drugs and folate deficiency
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alcohol, OC, phenytoin, methotrexate, trimethoprim, 5-fluorouracil
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Intestinal conjugase in folate metabolism
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inhibited by phenytoin
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Jejunal uptake of monoglutamate form of folate
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inhibited by alcohol and OC
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Dihydrofolate reductase
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inhibited by methotrexate, trimethoprim
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Thymidylate synthetase
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Thymidylate synthetase
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Folate deficiency
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MCC of increased serum homocysteine
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Labs in B12/folate deficiency
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pancytopenia; hypersegmented neutrophils; inc homocysteine
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Lab findings unique to B12 deficiency
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inc gastrin (PA), inc methylmalonic acid
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B12 reabsorbed/absorbed after administration of intrinsic factor
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pernicious anemia
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B12 reabsorbed/absorbed after administration of antibiotics
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bacterial overgrowth
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B12 reabsorbed/absorbed after administration of pancreatic extract
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chronic pancreatitis
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Acute blood loss
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initially normal Hb and Het; 0.9% saline uncovers RBC deficit
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Aplastic anemia caused by:
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drugs (e.g., phenylbutazone); infection (e.g., parvovirus); benzene
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Lab findings in aplastic anemia
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pancytopenia; hypocellular bone marrow
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Anemia in renal disease
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normocytic; decreased EPO
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Extravascular hemolysis
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macrophage phagocytosis of RBCs; inc unconjugated bilirubin and urine UBG
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Intravascular hemolysis
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dec serum haptoglobin; hemoglobinuria; hemosiderinuria
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Congenital spherocytosis
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AD; defect in spectrin; extravascular hemolysis; splenomegaly
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Blood findings in spherocytosis
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normocytic anemia; dense RBCs, inc MCHC, inc osmotic fragility
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Paroxysmal nocturnal hemoglobinuria
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missing decay accelerating factor; complement destruction RBCs, neutrophils, platelets
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S/S Paroxysmal nocturnal hemoglobinuria
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pancytopenia; hemoglobinuria; positive sugar water test and acidified serum test
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HbSS
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AR; missense mutation (valine for glutamic acid 6th positive (3-globin chain)
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Causes of sickling
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inc deoxyhemoglobin (hypoxemia, acidosis); HbS > 60%
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HbF
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inhibits sickling; hydroxyurea inc HbF
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Pathophysiology HbSS
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vasoocclusive crises, hemolytic anemia (extravascular)
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HbSS in children
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dactylitis (6-9 mths); Streptococcus pneumoniae sepsis (dysfunctional spleen)
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HbSS and osteomyelitis
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Salmonella paratyphi
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HbSS complications
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aplastic crisis (parvovirus), acute chest syndrome, autosplenectomy, calcium bilirubinate gallstones, priapism, aseptic necrosis
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HbAS
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microhematuria from sickling in renal medulla; renal papillary necrosis
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Hb electrophoresis
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HbAS- HbA 55-60%, HbS 40-45%; HbSS- HbS 90-95%, HbF 5-10%
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Blood findings in HbSS
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sickle cells; target cells; Howell-Jolly bodies (nuclear remnants)
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G6PD deficiency
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XR; oxidant damage (peroxide) to Hb (e.g., primaquine; dapsone; fava beans)
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Blood findings G6PD deficiency
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Heinz bodies (denatured Hb; special stain); bite cells
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Pyruvate kinase deficiency
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dec ATP; RBCs dehydrated; inc 2,3-BPG (right-shifted OBC)
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Warm type Autoimmune hemolytic anemia
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IgG; extravascular hemolysis; e.g, SLE, drugs
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Cold type Autoimmune hemolytic anemia
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IgM intravascular hemolysis; e.g., CLL, Mycoplasma
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Penicillin and hemolysis
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IgG antibody against penicillin attached to RBC (type II hypersensitivity)
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Methyldopa and hemolysis
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drug alters Rh antigens; IgG antibody against Rh antigens (type II hypersensitivity)
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Quinidine and hemolysis
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drug-IgM IC; intravascular hemolysis; type III hypersensitivity
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Lab findings in autoimmune hemolytic anemia
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positive direct Coombs'; spherocytes
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Micro-macroangiopathic hemolysis
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mechanical damage causing intravascular hemolysis
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Causes of micro/macro hemolysis
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aortic stenosis (MCC), DIC, TTP, HUS
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Peripheral blood findings in micro/macro hemolysis
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schistocytes; iron deficiency from hemoglobinuria
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Malaria and hemolysis
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intravascular hemolysis correlates with fever; falciparum- ring forms and gametocytes
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Leukemoid reaction
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exaggerated WBC response to infection; usually due to infection
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Leukoerythroblastic reaction
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marrow infiltrative disease peripheralizes myeloblasts/nucleated RBCs
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Causes of leukoerythroblastic reaction
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bone metastasis MCC, myelofibrosis
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Eosinophilia
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type I hypersensitivity (e.g., penicillin reaction); invasive helminthic infection
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Helminths not producing eosinophilia:
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pinworms, adult worms in ascariasis
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Atypical lymphocytes
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mononucleosis; CMV; toxoplasmosis; viral hepatitis; phenytoin
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Mononucleosis
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due to EBV; EBV attaches to CD21 receptors on B cells
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Clinical findings in mono
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exudative tonsillitis, generalized lymphadenopathy, hepatosplenomegaly
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Lab findings in mono
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atypical lymphocytosis; IgM heterophile antibodies against horse RBCs
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Lymphopenia
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T cell deficiencies (HIV); combined B/T deficiency (adenine deaminase deficiency)
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Lymphocytosis
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viral infections, whooping cough
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Corticosteroids and WBC
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lymphopenia, eosinopenia, neutrophilia
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Chronic Myeloproliferative disorder
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neoplastic stem cell disorder; splenomegaly; marrow fibrosis; risk for leukemia
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Examples of myeloproliferative disorder
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polycythemia vera, myelofibrosis and myeloid metaplasia
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Relative polycythemia
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plasma volume; inc RBC count; normal RBC mass
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Absolute polycythemia
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inc RBC count and RBC mass
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Appropriate polycythemia
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hypoxic stimulus for EPO to generate RBCs
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Causes of appropriate absolute polycythemia
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lung disease, cyanotic heart disease, high altitude
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Appropriate absolute polycythemia
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normal plasma volume; inc RBC mass; dec Sa02; inc EPO
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Inappropriate absolute polycythemia
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no hypoxic stimulus for EPO
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Causes of inappropriate polycythemia
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ectopic secretion EPO, polycythemia vera
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Polycythemia vera
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inc plasma volume and RBC mass; normal Sa02; dec EPO
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Ectopic EPO (renal cell carcinoma)
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normal plasma volume; inc RBC mass; normal Sa02; inc EPO
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Myelofibrosis myeloid metaplasia
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marrow fibrosis; extramedullary hematopoiesis; splenomegaly
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Lab findings in myelofibrosis
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tear drop RBCs; dry bone marrow aspirate (marrow fibrosis)
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Essential thrombocythemia
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MPD with increase in abnormal appearing platelets
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Myelodysplastic syndrome
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severe anemia in elderly; 30% develop leukemia; ringed sideroblasts
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Benzene causes
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aplastic anemia; acute leukemia
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Leukemia by age
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ALL: newborn-14; AML: 15-60; CML: 40-60; CLL: > 60
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Acute vs chronic leukemia
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acute: blasts > 30% in bone marrow; chronic: blasts < 10% in bone marrow
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AML
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Auer rods in myeloblasts
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Acute promyelocytic leukemia
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t(15;17); defect in retinoic acid; Rx retinoic acid (T maturation); DIC
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Acute monocytic leukemia
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gum infiltration
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CML
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t(9;22) of ABL POC; Philadelphia chromosome 22; dec leukocyte alkaline phosphatase score
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ALL
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early pre-B (80%); CALLA (CD10) and TdT positive; CNS and testicle involvement
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ALL
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t(12;21) offers good prognosis
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CLL
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B cell neoplasm; dec gamma-globulins; MCC generalized lymphadenopathy patients > 60-yrs-old
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Adult T cell leukemia
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HTLV-1; CD4 T cells; skin infiltration; lytic bone lesions with hypercalcemia
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Hairy cell leukemia
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positive TRAP stain; splenomegaly; Rx with purine nucleosides
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Nodal sites of cells
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germinal follicles: B cells; paracortex: T cells; sinuses: histiocytes
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Testicular cancer
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metastasizes to para-aortic nodes
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Stomach cancer
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metastasizes to left supraclavicular nodes (Virchow node)
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Phenytoin and peripheral smear
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atypical lymphocytosis
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Cat scratch disease
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Bartonella henselae; granulomatous microabscesses
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Follicular B-cell lymphoma
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t(14;18); overexpression of BCL-2 anti-apoptosis gene
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Burkitt lymphoma
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t(8; 14); EBV association; common childhood NHL; "starry sky" appearance
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Extranodal lymphomas: risk factors
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H. pylori (stomach); Sjogren's syndrome
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Mycosis fungoides
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CD4 T neoplasm; skin lesions with Pautrier's microabscesses
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Sezary syndrome
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leukemic phase of mycosis fungoides
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Polyclonal gammopathy
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sign of chronic inflammation
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Monoclonal gammopathy
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M component (spike); sign of plasma cell disorder
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Confirmatory tests for leukemia
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serum and urine immunoelectrophoresis; bone marrow aspirate
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Bence Jones protein
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light chains in urine; predictive of a malignant plasma cell disorder
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Multiple myeloma
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M spike; lytic bone lesions; pathologic fractures; hypercalcemia; renal failure
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MGUS (monoclonal gammapathy of undetermined significance)
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MC monoclonal gammopathy; may progress to myeloma
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Findings in MGUS (monoclonal gammapathy of undetermined significance)
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elderly patient; no BJ protein; no malignant plasma cells
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Waldenstrom's macroglobulinemia
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lymphoplasmacytic lymphoma; IgM M spike; hyperviscosity
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Hodgkin's lymphoma
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neoplastic component Reed Sternberg (RS) cell; CD 15 CD30 positive
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Lymphocyte predominant Hodgkin's
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infrequent classic RS cells
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Nodular sclerosing Hodgkin's
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female dominant supraclavicular nodes + anterior mediastinal nodes
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Mixed cellularity Hodgkin's
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male dominant; numerous RS cells; EBV association
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Hodgkin's prognosis
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stage of disease and type of Hodgkins most important factors
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Alkylating agents in Rx of Hodgkins
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inc risk for second malignancies (leukemia; NHL)
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Langerhan's histiocytes
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CD1 positive; Birbeck granules
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Letterer-Siwe disease
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malignant histiocytosis < 2-yrs-old; diffuse eczematous rash; organ involvement
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Hand-Christian-Christian disease
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malignant; lytic skull lesions, diabetes insipidus, exophthalmos
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Eosinophilic granuloma
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benign histiocytosis; lytic bone lesions with pathologic fractures
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Mast cells
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release histamine (pruritus; swelling); metachromatic granules positive with toluidine blue
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Urticaria pigmentosum
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localized mastocytosis; skin lesions swell and itch with scratching
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Amyloid
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twisted beta-sheet; apple green birefringence
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Primary amyloidosis
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AL amyloid derived from light chains; plasma cell disorders
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Secondary amyloidosis
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AA amyloid derived from serum-associated amyloid; chronic infections
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Alzheimer's disease
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amyloid precursor protein gene product chromosome 21; amyloid-beta
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Gaucher's disease
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macrophages have fibrillary appearance; deficiency glucocerebrosidase
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Niemann Pick's disease
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macrophages have soap bubble appearance; deficiency sphingomyelinase
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Hypersplenism
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splenomegaly; peripheral blood cytopenias; portal hypertension MCC
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Splenic dysfunction
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Howell Jolly bodies; susceptible to Streptococcus pneumoniae sepsis
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Anticoagulants
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tissue plasminogen activator, heparin, PGI2, ATIII, protein C/S
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Heparin MOA
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enhances ATIII activity (neutralizes all factors except V, VIII, fibrinogen)
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Protein C/S
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neutralize V and VIII
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Procoagulants
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coagulation factors, thromboxane A2 (platelet aggregation, vasoconstrictor)
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Protein C and S
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inactivate factors V and VIII; enhance fibrinolysis
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Von Willebrand factor
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complexes with factor VIII to enhance VIII:C activity; platelet adhesion
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Platelets
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receptors for von Willebrand factor and fibrinogen; synthesize thromboxane A2
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GpIb
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platelet receptor for von Willebrands factor
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GpIIb:IIIa
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platelet receptor for fibrinogen
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Extrinsic system factor
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VII
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Intrinsic system factors
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XII, XI, IX, VIII
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Final common pathway factors
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X, V, prothrombin (II), fibrinogen (I)
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Factor XIII
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cross-links insoluble fibrin; strengthens fibrin clots
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Vitamin K-dependent factors
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prothrombin, VII, IX, X, protein C and S
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Factors consumed in a clot
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fibrinogen, prothrombin, V, VIII; fluid is called serum
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Plasmin
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cleaves fibrinogen and insoluble fibrin into degradation products
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Bleeding time
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Bleeding time
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Aspirin and bleeding
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MCC of a prolonged bleeding time
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Tests for vWF
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ristocetin cofactor assay; vWF antigen assay; agar electrophoresis
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PT (prothrombin time)
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evaluates extrinsic pathway to fibrin clot
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PTT (partial thromboplastin time)
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evaluates intrinsic pathway to stable fibrin clot
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Fibrinolysis tests
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fibrin(ogen) degradation products; D-dimers (cross-linked insoluble fibrin)
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S/S platelet dysfunction
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cannot form temporary plug; epistaxis; petechiae; bleeding from scratches
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Idiopathic thrombocytopenic purpura (ITP)
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children; antibodies against GpIIb:IIla; no splenomegaly
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Chronic autoimmune thrombocytopenic purpura
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SLE; antibodies against GpIIb:IIIa receptors
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Heparin and platelets
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thrombocytopenia due to IgG antibody against heparin attached to PF4 on platelets
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PF4
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heparin neutralizing factor
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HIV hematologic complication
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thrombocytopenia MC hematologic abnormality; similar to ITP
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TTP
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platelet thrombi develop in areas of endothelial damage in small vessels; consumption of platelets
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S/S of TTP
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fever, thrombocytopenia, renal failure, hemolytic anemia with schistocytes, CNS deficits
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Lab findings in TTP
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thrombocytopenia, prolonged bleeding time, normal PT and PTT
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HUS
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similar to TTP; endothelial injury from Shiga-like toxin of 0157:H7 E. coli in undercooked beef
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S/S of factor deficiency
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no stable fibrin clot- late rebleeding; menorrhagia; GI bleeding; hemarthroses
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Hemophilia A
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XR; hemarthroses; prolonged PTT, dec factor VIII activity, normal VIII antigen
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Von Willebrand's disease
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AD; platelet adhesion defect + factor VIII deficiency
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Lab findings in VWD
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dec vWF, VIII antigen, and VIII:C; prolonged bleeding time
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Desmopressin acetate
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Rx of choice for mild von Willebrand's disease and hemophilia A
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Circulating anticoagulants
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antibodies destroy coagulation factors
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Lab finding in circulating anticoagulant
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prolonged PT and/or PTT corrected with mixing studies
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Vitamin K deficiency
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dec epoxide reductase activity (dec function vitamin K); hemorrhagic diathesis; inc PT
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Causes of vitamin K deficiency
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antibiotics MC, newborn, malabsorption, warfarin
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DIC
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activation coagulation system from release of tissue thromboplastin and/or endothelial cell damage
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DIC
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consumption coagulation factors by fibrin clots; patient also anticoagulated
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causes of DIC
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septic shock MCC, rattlesnake bite, massive trauma, amniotic fluid
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S/S of DIC
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bleeding from all scratches, holes, needle sites
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Lab findings in DIC
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thrombocytopenia, inc PT and PTT, D-dimers (best test), anemia
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Antiphospholipid antibodies
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lupus anticoagulant and anticardiolipin antibodies; vessel thrombosis
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Warfarin
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inhibits epoxide reductase; PT best test but PTT also prolonged
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Warfarin
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full anticoagulation in 3 days when gamma-carboxylated prothrombin disappears
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Warfarin
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ingredient in rat poison; danger to children in households with grandparents on warfarin
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Rx for warfarin overanticoagulation
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intramuscular vitamin K (6-8 hrs), fresh frozen plasma (immediate)
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Heparin
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enhances ATIII; PTT best test but PT also prolonged
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oral contraceptives
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estrogen inc coagulation factor synthesis and 4- ATIII; predisposes to thrombosis
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Factor V Leiden
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MC hereditary thrombosis; resistant to degradation by protein C/S
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ATIII deficiency
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no prolongation of PTT with administration of heparin
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Hemorrhagic skin necrosis
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post-warfarin therapy in patient with heterozygote protein C deficiency
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M cells
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specialized cells that transfer foreign antigens to lymphocytes in Peyer's patches
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Blood group O
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some patient have anti-AB-IgG antibodies; increased incidence duodenal ulcers
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Blood group A
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increased incidence of gastric carcinoma
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newborns and blood group
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do not have natural blood group antibodies at birth (e.g., anti-A-IgM)
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Elderly and blod groups
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may lose natural blood group antibodies; no hemolytic reaction to mismatched blood
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Rh antigens
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inherited in autosomal codominant fashion; Rh antigens include D, C, c, E, e
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Atypical antibodies
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antibodies against Rh or non-Rh blood group antigens (e.g., anti-D)
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Duffy antigen
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receptor for Plasmodium vivax; blacks often lack
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Duffy antigen
Antibody screen |
indirect Coomb's test; detects atypical antibodies in serum
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Cytomegalovirus
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MC infection transmitted by blood transfusion; MC antibody
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MCC of post-transfusion hepatitis
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Hepatitis C
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Major crossmatch
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patient serum reacted against donor RBCs; does not guarantee RBC survival
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Universal donor
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blood group O; no antigens on the surface of RBCs
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Universal recipient
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blood group AB; no natural blood group antibodies in serum
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Packed RBC transfusion
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raises Hb by 1 gm/dL and Hct by 3%
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Cryoprecipitate
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fibrinogen and factor VIII
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Fresh frozen plasma
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replacement for multiple factor deficiencies (e.g., cirrhosis, DIC)
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Allergic transfusion reaction
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type I IgE-mediated hypersensitivity reaction
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Febrile transfusion reaction
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recipient anti-HLA antibodies react against donor leukocytes
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Intravascular HTR
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transfusion of ABO incompatible blood (e.g., A person receives B blood)
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Extravascular HTR
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antibody attaches to donor RBCs; macrophage phagocytosis and hemolysis
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Positive direct Coomb's test
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present in both types of hemolytic transfusion reactions
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S/S of hemolytic transfusion reaction
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jaundice, no increase in Hb, hemoglobinuria
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ABO Hemolytic disease of the newborn
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mother O and baby A or B; transplacental passage of maternal anti-AB-IgG
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ABO Hemolytic disease of the newborn
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positive direct Coomb's test; spherocytes; MCC unconjugated hyperbilirubinemia first 24-hrs
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Rh Hemolytic disease of the newborn
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mother Rh (D antigen) negative and fetus Rh (D antigen) positive
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Rh Hemolytic disease of the newborn
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no hemolysis in first Rh incompatible pregnancy
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Rh Hemolytic disease of the newborn
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maternal anti-D crosses placenta; potential for hydrops fetalis; high risk for kernicterus
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Rh immune globulin
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anti-D; coats D antigen site on fetal RBCs in maternal circulation
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Rh Hemolytic disease of the newborn lab
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positive direct Coomb's; severe anemia and hyperbilirubinemia
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ABO Hemolytic disease of the newborn
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protects mother from Rh sensitization (development of anti-D antibodies)
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O Rh negative mother with A Rh positive baby
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A+ cells destroyed by mothers anti A-IgM
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Blue fluorescent light
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converts unconjugated bilirubin in skin into harmless water soluble dipyrrole
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