Approach to Bleeding Disorders Evaluation of the patient

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 – a. PTT – platelet count – fibrinogen – thrombin time

Specific Laboratory Tests • Mixing studies – patient and PNP mixed 1: 1 – incubated 2 hours at 37 o 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 • • • a. PTT 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 50 cc 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 +/- a. PTT – 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