Survey
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
Download approach to bleeding disorders
Document related concepts
Transcript
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) – 0.3 micrograms/kg IV in 50cc NS over 30 minutes – intranasally 2 puffs for adults, 1 puff for children • Factor VIII product containing Vwf – Humate P – Koate HP – Alphanate • Cryoprecipitate ONLY IF VWF/VIII PRODUCT NOT AVAILABLE! – 1 bag/10 kg q 12 to 24 hours depending upon the bleeding • epsilon amino caproic acid (Amicar) Other Congenital Defects • Other Factor deficiencies • Platelet defects – – – – very rare platelet aggregation studies electron microscopy bleeding time What else could it be? • Vitamin K deficiency – drug-induced/malabsorption – rarely nutritional in an outpatient • Liver Disease – long PT +/- aPTT – poor clearance of coagulation products • DIC Liver Disease • • • • Decreased synthesis of factors Synthesis of abnormal factors Increased fibrinolysis Thrombocytopenia Liver Disease • Fresh frozen plasma – replete factors – WILL NOT CORRECT THE PT • Cryoprecipitate – fibrinogen • Platelets Disseminated Intravascular Coagulation Treat the underlying cause Disseminated Intravascular Coagulation • Replete deficient factors – FFP – cryoprecipitate – platelets • Role of heparin? Don’t Forget! Factor XIV deficiency (insufficient suture) Drug Treatments • • • • • Stop causative/contributory medications Vitamin K or C DDAVP epsilon amino caproic acid (Amicar) Topical procoagulants Bone Marrow Diseases • Acute leukemias • Myelodysplasia • Myeloproliferative disorders – P. vera – dysfunctional platelets Tests are normal-Now what? • • • • • • • simple purpura senile purpura Factor XIII deficiency alpha-2-antiplasmin deficiency mild factor deficiency amyloidosis vascular disorders Still more? • • • • • Hereditary hemorrhagic telangiectasia scurvy Ehlers-Danlos syndrome? Henoch-Schonlein purpura the un-diagnosable fibrinolytic defect Summary • History & Physical Examination • Laboratory tests – screening tests – specific diagnostic tests • Diagnosis-specific therapy – Factor replacement – Drugs Question #1 The patient with normal laboratories, dry IV sites, and blood gushing out a surgical drain probably has: a. von Willebrand’s disease b. undiagnosed hemophilia c. mechanical bleeding d. a bad attitude Question #2 Four units of FFP will completely correct the PT in a patient on warfarin in all but the largest of patients. True False
