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Hemostasis Objectives
- -Vitamin K deficiency
- -Von Willebrand's Disease (vWB)
- -Idiopathic Thrombocytopenia Purpura (ITP)
- -Thrombotic Thrombocytopenia Purpura (TTP)
- -Hemolytic-Uremic Syndrome (HUS)
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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)
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Vitamin K deficiency
- -Replacement: oral Vitamin K 5-10 mg PO daily x 3 days
- -Chronic: MVI with vit K daily.
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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
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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)
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Cryoprecipitate (CRYO)
- -acquired deficiencies. due to DIC or thrombolytic therapy, congential hypofibrinogenemia or dysfibrinogenemia.
- -CRYO- only source of concentrated fibrinogen
- -Horizon: RiaSTAT.
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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).
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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)
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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.
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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.
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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.
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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.
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ITP Treatment Rationale
- -platelet transfusions not usually given unless pt acutely bleeding.
- -therapy aimed at eliminating antibodies.
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ITP Treatment Options
- -Glucocorticoids (Prednisone)
- -IV Ig= more intermediate effect for severe thrombocytopenia/bleeding.
- -Splenectomy
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ITP second line agents
- -Vinca alkaloids
- -Danazol
- -Rituximab (monoclonal antibody)
- -Immunosuppressive agents
- -WinRho (RhoD positive patients)
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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.
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TTP Treatment options
- -Plasmapheresis
- -Steroids
- -Rituximab
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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.
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HUS Treatment
- -symptomatic and supportive:
- *pressors
- *antibiotics
- *dialysis/transplant
- -plasmapheresis
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