Approach to Bleeding Disorders

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