1. Ig that crosses placenta
  2. Effects of bradykinin
    • Vasodilation
    • Increased permeability
    • Increased pain
  3. XIIa is the beginning of the intrinsic coag pathway. It also activates:
    • Kallikrein, which causes formation of:
    • bradykinin
    • plasmin
  4. Antithrombin inhibits
    Thrombin (II), IXa, Xa, XIa, XIIa
  5. Acanthocyte (spur cell) seen in
    • Liver disease
    • abetalipoproteinemia
  6. Basophilic stippling of RBCs is seen in
    • Lead poisoning
    • thalassemias
    • ACD
    • Iron def
  7. Bite cell is seen in
    G6PD deficiency
  8. Target cells are seen in
    • HbC disease
    • asplenia
    • liver disease
    • thalassemia
  9. Heinz bodies are composed of ___ and lead to ___
    • Denatured hemoglobin due to oxidized (ferric) iron
    • Bite cells
  10. Heinz bodies are seen in
    • alpha-thal
    • G6PD deficiency
  11. Howell-Jolly bodies are ___ and are seen in ___
    • Basophilic nuclear remnants in RBCs
    • asplenia
  12. Sideroblastic anemia indicates
    Defect in heme synthesis
  13. Hereditary sideroblastic anemia
    • X-linked defect in delta-ALA synthase
    • Tx with B6
  14. Reversible etiologies of sideroblastic anemia
    • Alcohol
    • lead
  15. Hepcidin: what disease and why is it important?
    • ACD
    • increases with inflammation and causes a decrease release of iron from macrophages
  16. G6PD deficiency causes a decrease in
  17. HbC defect
    Beta chain mutation but milder than sickle cell
  18. Paroxysmal nocturnal hemoglobinuria pathogenesis
    • Intravascular hemolysis due to complement-mediated lysis
    • Impaired synthesis of GPI anchor/decay accelerating factor in RBC membrane
    • Increase in urine hemosiderin
  19. Warm agglutinin: Ig and associations
    • IgG
    • SLE, CLL, some drugs
  20. Cold agglutinin: Ig and associations
    • IgM
    • Mycoplasma pneumoniae or mono
  21. Ferritin is high in
    • ACD
    • Hemochromatosis
  22. Ferritin is low in
    Iron def
  23. Transferrin or TIBC high in
    • iron deficiency
    • preg/OCPs
  24. Transferrin or TIBC low in
    • ACD
    • hemochromatosis
  25. Lead poisoning affected enzymes
    • Ferrochelatase
    • ALA dehydratase
  26. Lead poisoning accumulated substrate
    Protoporphyrin in the blood
  27. Acute intermittent porphyria affected enzyme
    • Porphobilinogen deaminase
    • AKA uroporphyrinogen-I-synthase
  28. Acute intermittent porphyria accumulated substrate
    • Porphobilinogen
    • delta-ALA
    • uroporphyrin (urine)
  29. Porphyria cutanea tarda affected enzyme
    Urophorphyrinogen decarboxylase
  30. Porphyria cutanea tarda accumulated substrate
    Uroporphyrin (tea-colored urine immediately)
  31. Rate-limiting heme synth step: enzyme, cofactor, an deficiency
    • delta-ALA synthase
    • needs B6
    • dysfunction gives you X-linked sideroblastic anemia
  32. Lead poisoning treatment
    • Dimercaprol and EDTA
    • Succimer for kids
  33. Bernard-Soulier disease
    Decreased Gp1b means less platelet-collagen (vWF) adhesion
  34. Glanzmann's thrombasthenia
    Less GpIIb/IIIa means defect in platelet-platelet aggregation
  35. The antibody in ITP
  36. Deficiency in TTP
    • Def of ADAMTS 13 decreases vWF degredation
    • Too much vWF increases platelet agg and thrombosis
  37. TTP labs and symptoms
    • schistocytes and high LDH
    • Neuro, renal, fever, hemolytic anemia
  38. von Willebrand's disease
    less vWF means defective VIII protection and defective platelet-collagen adhesion
  39. Factor V Leiden
    Mutant factor V can't be degraded by protein C
  40. Prothrombin gene mutation: where and what?
    • 3' UTR
    • Venous clots
  41. Protein C or S deficiency
    Less inactivation of V and VIII means clotting
Card Set
USMLE Step 1 heme from FA 2010