Hemostasis Hematology

  1. Hemostasis Objectives
    • -Vitamin K deficiency
    • -Von Willebrand's Disease (vWB)
    • -Idiopathic Thrombocytopenia Purpura (ITP)
    • -Thrombotic Thrombocytopenia Purpura (TTP)
    • -Hemolytic-Uremic Syndrome (HUS)
  2. Management of Bleeding Complications
    • -Minor: hold warfarin, PO vit K if INR reversal required
    • -Serious: hold warfarin, IV vit K, supplement with FFP, factor 7a, or prothrombin complex concentrates
    • -Life-threatening: reverse prolonged INR with IV vit K and with factor 7a or prothrombin complex concentrates (PCC's)
  3. Vitamin K deficiency
    • -Replacement: oral Vitamin K 5-10 mg PO daily x 3 days
    • -Chronic: MVI with vit K daily.
  4. Vitamin K Clinical PEARLS
    • -mildly elevated INR without bleeding- VK not always indicated.
    • -little bit is usually enough
    • -minor bleeding: oral Vit K
    • -major bleeding: IV vit K
    • -Avoid SC/IM administration
  5. Fresh Frozen Plasma (FFP)
    • -liver dx, Vit K deficiency, DIC, immediate short term reversal of over-anticoagulation with Coumadin.
    • -1/2 life- 3-5 hours. factor 7 difficult to replace without volume overload. Vit K AND FFP indicated in patients with high INR and bleeding.
    • -FFP not indicated unless PT or PTT is >1.5 x the mean of normal range. (not for mildly prolonged PT or PTT)
  6. Cryoprecipitate (CRYO)
    • -acquired deficiencies. due to DIC or thrombolytic therapy, congential hypofibrinogenemia or dysfibrinogenemia.
    • -CRYO- only source of concentrated fibrinogen
    • -Horizon: RiaSTAT.
  7. Prothrombin Complex Concentrates
    • -Profilnine- non-activated Factor 9, 2, 10 and low level of factor 7.
    • -FEIBA- non-activated Factor 2, 9, and 10, and Factor 7a (activated).
  8. NovoSeven (rFVIIa)
    • -alternative for pts who don't respond to other replacement methods
    • -major bleeding associated w/ warfarin overdose or hepatic failure who require fast hemostasis
    • -$$$$$$
    • -must have stores of 10, 2, and 1 (common pathway)
  9. Von Willebrand Disease
    • -defect of primary hemostasis (platelets)
    • -qualitative vs quantitative deficiency of VWF.
    • -frequency of mild vWD 1 in 200
    • -symptoms depend on severity.
  10. Von Willebrand Factor
    • -bridge between platelets and subendothelium
    • -bridge between platelets
    • -carrier protein for factor 8.
    • -defects quantitative (type 1,3), qualitative (type 2)
    • -defects result in decreased platelet aggregation and adhesion.
  11. vWD therapeutic management
    • -Desmopressin (DDAVP) increases circulating vWF. useful for minor bleeding in type 1.
    • -Factor VIII preps: intermediate-purity factor 8. used for major bleeding and patients with type 2 and 3.
    • -vWD is dosed on RISTOCETIN COFACTOR activity.
  12. Idiopathic Thrombocytopenic Purpura (ITP)
    • -autoimmune antibodies form and alter platelets.
    • -platelet removal by spleen increased
    • -unknown cause
    • -increased bone marrow production, can't keep up with destruction.
  13. ITP Treatment Rationale
    • -platelet transfusions not usually given unless pt acutely bleeding.
    • -therapy aimed at eliminating antibodies.
  14. ITP Treatment Options
    • -Glucocorticoids (Prednisone)
    • -IV Ig= more intermediate effect for severe thrombocytopenia/bleeding.
    • -Splenectomy
  15. ITP second line agents
    • -Vinca alkaloids
    • -Danazol
    • -Rituximab (monoclonal antibody)
    • -Immunosuppressive agents
    • -WinRho (RhoD positive patients)
  16. Thrombotic Thrombocytopenic Purpura (TTP)
    • -result of lack of enzyme (ADAMSTS13) responsible for cleaving vw factor.
    • -causes formation of many small blood clots
    • -high number of platelets consumed- decreased platelet count
    • -Schistocytes on blood film.
  17. TTP Treatment options
    • -Plasmapheresis
    • -Steroids
    • -Rituximab
  18. Hemolytic-Uremia Syndrome (HUS)
    • -related to TTP
    • -caused by E. coli infections
    • -Hemolysis, microthrombi to brain and kidneys
    • -most common- infants, young children, pregnant women.
  19. HUS Treatment
    • -symptomatic and supportive:
    • *pressors
    • *antibiotics
    • *dialysis/transplant
    • -plasmapheresis
Card Set
Hemostasis Hematology