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Disorders of hemostasis

Thrombocytopenias and Coagulopathies

Eileen Gaspar

on 22 July 2010

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Transcript of Disorders of hemostasis

Gap plugged by platelets
Vasoconstriction Coagulation cascade Congenital Osler Weber Rendu Ehrlos Danlos Hereditary hemorhagic telengiectasias
Familial telengiectasias of fingers, nasal & oral mucosa
Hx nosebleeds
Pulmonary htn Decreased production/marrow destruction Excess destruction Decreased platelet fcn Drugs Radiotherapy Megaloblastic anemia
Aplastic anemia
Autoimmune Consumptive Membrane defects Acquired Vasc endothelial defect Congenital Drugs Post transfusion purpura ITP SLE CLL HIV 80% pts respond to prednisone almost always w/in 3 wks
Dx of exclusion
If > 60 yo, do BONE MARROW to r/o myelofibrosis DIC TTP HUS HELLP Heparin, quinidine, rifampin, sulfonamide combinations, digoxin, acetominophen Bernard Soulier absent gpIb
Glanzmann absent gIIb-IIIa Plavix VWF mildly prolonged PTT ASA, NSAIDs
Myeloprolif Uremia (Inherited thrombocytopenic disorder -
decreased platelet count
Giant platelets
Fam hx low platelets
Refractory to corticosteroids
Congenital Acquired Factor VIII & IX def
NO NSAIDS, IM injxn, DDAVP Liver: decreased clotting factor synthesis, absorption of Vit K, abnormal platelet fcn Malapsorption - less uptake of Vitamin K Anticoagulants DIC Vit K def Acquired Senile purpura Infection Meningococcus Measles Dengue hemorrhagic HSP When picking best for pts w/ VTE and met CA, pick LMWH > warfarin Delayed-onset HIT => 3-4 wks after heparin exposure, tx w/ direct thrombin inhibitor
Allpts with HIT even w/o thrombosis should get direct thrombin inhibitor (argatroban for w/ & w/o, lepirudin for w/ thrombosis only) Thrombotic Disorders Inherited Factor V Leiden:
heterzygosity - increases lifetime risk for clot 7 fold
homozygosity - increases lifetime risk 20 fold
no ppx needed for asx pregnant women
risk for recurrent thrombosis is not greater than those w/o underlying thrombophilic abnormality Acquired Pregnancy
reduced levels of protein S
don't test for thrombophilic d/o or antiphospholipid ab syndrome at dx of acute thrombosis
women w/ at least 1 unexplained fetal loss in T2 or T3 esp w/ placental infarction-test for thrombophilic defect Anemia & thrombocytopenia
Tx plasma exchange & plasma infusion if plasmapharesis not available
NO PLATELETS Incidental thrombocytopenia of pregnancy
ddx 3rd trimester - HELLP, HUS, TTP, ITP
if <70 K, be concerned
does not affect fetal platelet count Estrogen use (OCPs)
low risk for recurrent VTE Antiphosphlipid ab Syndrome
+ unprovoked venous thrombotic events => long-term oral anticoagulation
goal INR 2-3
remember have to be positive more than once and do not repeat test while still on warfarin
Nephrotic syndrome - losing anticoagulants Prothrombin gene mutation G20210A
Antithrombin III def
Protein C and S def hypermobility - hyperelasticity
joint dislocation skin scarring
vascular rupture
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