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Comprehensive Approach to Bleeding Disorders Evaluation

Understand the patient's history, physical examination, and laboratory assessment for various types of bleeding disorders. Learn about specific tests, diagnosis, and treatment options.

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Comprehensive Approach to Bleeding Disorders Evaluation

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  1. Approach to Bleeding Disorders

  2. Evaluation of the patient • History • Physical Examination • Laboratory Evaluation

  3. History • Are you a bleeder? • surgical challenges • accidents & injuries • dental extractions • menstrual history

  4. Type of Bleeding • ecchymoses • petechiae • epistaxis • deep soft tissue bleed • hemarthroses • GI bleeding

  5. Does it sound genetic? • duration of bleeding history • congenital v. acquired • family history • examine pedigree • determine inheritance

  6. Medical History • liver disease • renal disease • malignancies • antibiotic therapy • poor nutrition (Vit. K or C)

  7. Physical Examination • current hemorrhage • nature and extent • intercurrent illnesses • liver disease • petechiae/ecchymoses

  8. Laboratory Assessment • Guided by history • Screening tests • PT • aPTT • platelet count • fibrinogen • thrombin time

  9. 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

  10. Circulating Anticoagulant • Lupus anticoagulant/APA syndrome • rarely have associated bleeding • tend to thrombose • Acquired factor inhibitors • Factor VIII most common • tertiary care referral

  11. 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

  12. 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

  13. von Willebrand’s Testing • aPTT • Factor VIII activity • von Willebrand’s Factor • Ristocetin Cofactor • von Willebrand’s Factor multimers

  14. 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

  15. 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)

  16. Other Congenital Defects • Other Factor deficiencies • Platelet defects • very rare • platelet aggregation studies • electron microscopy • bleeding time

  17. 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

  18. Liver Disease • Decreased synthesis of factors • Synthesis of abnormal factors • Increased fibrinolysis • Thrombocytopenia

  19. Liver Disease • Fresh frozen plasma • replete factors • WILL NOT CORRECT THE PT • Cryoprecipitate • fibrinogen • Platelets

  20. Disseminated Intravascular Coagulation Treat the underlying cause

  21. Disseminated Intravascular Coagulation • Replete deficient factors • FFP • cryoprecipitate • platelets • Role of heparin?

  22. Don’t Forget! Factor XIV deficiency (insufficient suture)

  23. Drug Treatments • Stop causative/contributory medications • Vitamin K or C • DDAVP • epsilon amino caproic acid (Amicar) • Topical procoagulants

  24. Bone Marrow Diseases • Acute leukemias • Myelodysplasia • Myeloproliferative disorders • P. vera • dysfunctional platelets

  25. Tests are normal-Now what? • simple purpura • senile purpura • Factor XIII deficiency • alpha-2-antiplasmin deficiency • mild factor deficiency • amyloidosis • vascular disorders

  26. Still more? • Hereditary hemorrhagic telangiectasia • scurvy • Ehlers-Danlos syndrome? • Henoch-Schonlein purpura • the un-diagnosable fibrinolytic defect

  27. Summary • History & Physical Examination • Laboratory tests • screening tests • specific diagnostic tests • Diagnosis-specific therapy • Factor replacement • Drugs

  28. 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

  29. 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

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