approachtobleedingdisorders.ppt.pptVIP

Approach to Bleeding Disorders Evaluation of the patient History Physical Examination Laboratory Evaluation History Are you a bleeder? surgical challenges accidents injuries dental extractions menstrual history Type of Bleeding ecchymoses petechiae epistaxis deep soft tissue bleed hemarthroses GI bleeding Does it sound genetic? duration of bleeding history congenital v. acquired family history examine pedigree determine inheritance Medical History liver disease renal disease malignancies antibiotic therapy poor nutrition (Vit. K or C) Physical Examination current hemorrhage nature and extent intercurrent illnesses liver disease petechiae/ecchymoses Laboratory Assessment Guided by history Screening tests PT aPTT platelet count fibrinogen thrombin time Specific Laboratory Tests Mixing studies patient and PNP mixed 1:1 incubated 2 hours at 37o C perform clotting assay as usual Uncorrected - circulating anticoagulant Corrected - factor deficiency Circulating Anticoagulant Lupus anticoagulant/APA syndrome rarely have associated bleeding tend to thrombose Acquired factor inhibitors Factor VIII most common tertiary care referral Factor deficiencies Hemophilia A or B Factor VIII or IX assays Probably mild unless bleeding patient is an infant male Send to Hemophilia Treatment Center von Willebrand’s disease most common genetic bleeding disorder many different types von Willebrand’s Disease autosomal dominant except Type III patients range from asymptomatic to spontaneous bleeding similar to a severe hemophiliac characterized by mucocutaneous bleeding von Willebrand’s Testing aPTT Factor VIII activity von Willebrand’s Factor Ristocetin Cofactor von Willebrand’s Factor multimers von Willebrand’s Disease Type I normal molecule in abnormally low quantities normal distribution of multimers Type II abnormal molecule abnormal distribution of multimers with decrease in the largest molecular weight forms Type III severe von Willebrand’s Disease - Treatment DDAVP (Stimate) 0.