shosh: heme

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  1. WBC with bilobate nucleus
  2. Cromolyn sodium
    • Prevents mast cell degranulation by stabilizing the cell membrane
    • Used to treat asthma
  3. Cell that produces histaminase and arylsulfatase
  4. CD14
    Monocyte and macrophage marker
  5. WBC with kidney-shaped nucleus
  6. NK cell markers
    CD16, CD56
  7. CD3
    T cell marker
  8. Class of antibodies to the A and B blood group markers
    IgM (do not cross placenta)
  9. Class of antibodies to the Rh blood group marker
    IgG (do cross placenta)
  10. Hemorrhagic disease of the newborn
    • Caused by vitamin K deficiency, common in premies
    • Treat with vitamin K injection
  11. Effect of factor X
    • Converts prothrombin to thrombin
    • Thrombin then converts fibrinogen to fibrin
    • Fibrin forms clots, stabilized by factor XIII and Ca
  12. Hemophilia A
    Factor VIII deficiency
  13. Hemophilia B
    Factor IX deficiency
  14. What activates bradykinin?
    Kallikrein, which is activated by factor XII
  15. vWF
    • Links platelet Ib receptor to subendothelial collagen
    • Binds factor VIII, protects it from degradation
  16. Ticlopidine, clopidogrel
    Inhibit ADP-induced expression of GPIIb/IIIa
  17. Abciximab
    Inhibits IIb/IIIa
  18. Epoxide reductase
    • Activates vitamin K, allows it to act as a cofactor in the carboxylation reaction needed for production of clotting factors
    • Inhibited by warfarin
  19. Protein C
    • Activated by protein S
    • Inactivates factors V and VIII
    • Factor V Leiden mutation produces a version of factor V that cannot be inactivated by protein C
  20. Basophilic stippling
    • Thalassemias
    • Anemia of chronic disease
    • Iron deficiency
    • Lead poisoning
  21. Bite cells and Heinz bodies
    G6PD deficiency
  22. Defective enzyme in sideroblastic anemia
    Ala synthase
  23. Target cell
    • HbC disease
    • Asplenia
    • Liver disease
    • Thalassemia
  24. Hepcidin
    • Hormone that regulates iron levels
    • Synthesized in liver
    • Acts on bone marrow macropages and intestinal epithelial cells
    • Inhibits iron transport, and mediates iron sequestration in anemia of chronic disease
  25. Folate deficiency
    • Folate is needed to convert homocysteine to methionine
    • Megaloblastic anemia with hypersegmented neutrophils and increased levels of homocysteine
  26. B12 deficiency
    • Megaloblastic anemia with hypersegmented neutrophils, increased homocysteine, and increased methalmalonic acid
    • Peripheral neuropathy
    • Can be due to malnutrition, malabsorption, pernicious anemia, or diphyllobothrium latum
  27. Fanconi's anemia
    • Caused by an inherited defect in DNA repair
    • Presents with short stature, increased incidence of cancer, and aplastic anemia
    • Often associated with AML
  28. Pyruvate kinase deficiency
    • Caused by an AR mutation
    • Inability to make ATP causes RBCs to burst from osmotic stress
    • Presents with hemolytic anemia in newborns
  29. PNH
    • Caused by impaired synthesis of GIP, which functions to anchor CD55 and CD59 to RBC membranes
    • CD55/59 prevent RBCs from getting destroyed in the spleen
    • Presents with intravascular hemolysis, due to complement-mediated RBC lysis
    • Labs show increased urine hemosiderin
    • Treat with eculizumab
  30. Warm agglutinin autoimmune hemolytic anemia
    • Mediated by IgG
    • RBC destruction in spleen
    • Seen in pxs with SLE, CLL, and on methyldopa
  31. Cold agglutinin autoimmune hemolytic anemia
    • Mediated by IgM
    • RBC destruction in liver
    • Attacks of acute anemia triggered by cold temperatures
    • Seen in patients with M. pneumonia or mono
  32. Schistocytes on peripheral blood smear
    Indicate mechanical destruction of RBCs
  33. Hemolytic uremic syndrome
    • Can be precipitated by E. coli infection
    • Characterized by hemolytic anemia, thrombocytopenia and renal failure
  34. Thrombotic thrombocytopenic purpura
    • Due to mutant ADAMTS-13 metalloproteinase
    • Failure to cleave vWF causes large vWF monomers to bounce around in the circulation and smash RBCs
    • Presents with a triad of neurological symptoms, renal failure, thrombocytopenia, fever, and microangiopathic hemolytic anemia
  35. Acute intermittent porphyria
    • Due to a porphobilinogen deaminase deficiency
    • Presents with abdo pain, pink urine, and polyneuropathies
    • Treat with glucose and heme to inhibit ala synthase
  36. Porphyria cutanea tarda
    • Due to a uroporphyrinogen decarboxylase deficiency
    • Most common porphyria
    • Presents with tea-colored urine and blistering photosensitivity
    • Exacerbated by alcohol
  37. B6 deficiency
    • B6 is needed to convert glycine and succinyl CoA into d-ala
    • Deficiency can be induced by isoniazid, and causes sideroblastic anemia
  38. Treatment for lead poisoning
    • Dimercaprol in adults
    • Succimer in children
  39. Bernard-Soulier disease
    • GP1b deficiency
    • Platelets cannot adhere to subendothelial collagen
  40. Glanzman's thrombasthenia
    • GP IIa/IIIb deficiency
    • Platelets cannot adhere to each other
    • Normal platelet count
    • Ristocetin triggers aggregation
    • ADP does not trigger aggregation
  41. ITP
    • Caused by autoantibodies against GP IIb/IIIa
    • Platelets are destroyed in the spleen
  42. Poor aggregation in response to ristocetin but normal aggregation in response to ADP
    Hemophilia B (factor IX deficiency)
  43. Most common cause of hereditary hypercoagulability
    • Factor V Leiden
    • Factor V cannot be degraded by protein C
  44. Prothrombin mutation
    • Mutation in 3 prime untranslated region
    • Associated with venous clots
  45. Lepirudin, bivalirudin, argatroban
    • Hirudin derivatives
    • Direct thrombin inhibitors
    • Use as an alternative to heparin in patients with HIT
  46. Aminocaproic acid
    Inhibit fibrinolysis, use to treat thrombolytic overdose
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shosh: heme
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