Approach to Bleeding Disorders April 4, 1998 Approach to Bleeding Disorders 1

Evaluation of the patient Approach to Bleeding April 4, 1998 Evaluation of the patient History Physical Examination Laboratory Evaluation 2

History Are you a bleeder? surgical challenges accidents & injuries Approach to Bleeding April 4, 1998 History Are you a bleeder? surgical challenges accidents & injuries dental extractions menstrual history 3

Type of Bleeding ecchymoses petechiae epistaxis deep soft tissue bleed Approach to Bleeding April 4, 1998 Type of Bleeding ecchymoses petechiae epistaxis deep soft tissue bleed hemarthroses GI bleeding 4

Does it sound genetic? duration of bleeding history Approach to Bleeding April 4, 1998 Does it sound genetic? duration of bleeding history congenital v. acquired family history examine pedigree determine inheritance 5

Medical History liver disease renal disease malignancies Approach to Bleeding April 4, 1998 Medical History liver disease renal disease malignancies antibiotic therapy poor nutrition (Vit. K or C) 6

Physical Examination current hemorrhage nature and extent Approach to Bleeding April 4, 1998 Physical Examination current hemorrhage nature and extent intercurrent illnesses liver disease petechiae/ecchymoses 7

Laboratory Assessment Approach to Bleeding April 4, 1998 Laboratory Assessment Guided by history Screening tests PT aPTT platelet count fibrinogen thrombin time 8

Specific Laboratory Tests Approach to Bleeding April 4, 1998 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 9

Circulating Anticoagulant Approach to Bleeding April 4, 1998 Circulating Anticoagulant Lupus anticoagulant/APA syndrome rarely have associated bleeding tend to thrombose Acquired factor inhibitors Factor VIII most common tertiary care referral 10

Factor deficiencies Hemophilia A or B von Willebrand’s disease Approach to Bleeding April 4, 1998 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 11

von Willebrand’s Disease Approach to Bleeding April 4, 1998 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 12

von Willebrand’s Testing Approach to Bleeding April 4, 1998 von Willebrand’s Testing aPTT Factor VIII activity von Willebrand’s Factor Ristocetin Cofactor von Willebrand’s Factor multimers 13

von Willebrand’s Disease Approach to Bleeding April 4, 1998 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 14

von Willebrand’s Disease - Treatment Approach to Bleeding April 4, 1998 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) 15

Other Congenital Defects Approach to Bleeding April 4, 1998 Other Congenital Defects Other Factor deficiencies Platelet defects very rare platelet aggregation studies electron microscopy bleeding time 16

What else could it be? Vitamin K deficiency Liver Disease DIC Approach to Bleeding April 4, 1998 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 17

Liver Disease Decreased synthesis of factors Approach to Bleeding April 4, 1998 Liver Disease Decreased synthesis of factors Synthesis of abnormal factors Increased fibrinolysis Thrombocytopenia 18

Liver Disease Fresh frozen plasma Cryoprecipitate Platelets Approach to Bleeding April 4, 1998 Liver Disease Fresh frozen plasma replete factors WILL NOT CORRECT THE PT Cryoprecipitate fibrinogen Platelets 19

Disseminated Intravascular Coagulation Approach to Bleeding April 4, 1998 Disseminated Intravascular Coagulation Treat the underlying cause 20

Disseminated Intravascular Coagulation Approach to Bleeding April 4, 1998 Disseminated Intravascular Coagulation Replete deficient factors FFP cryoprecipitate platelets Role of heparin? 21

Factor XIV deficiency (insufficient suture) Approach to Bleeding April 4, 1998 Don’t Forget! Factor XIV deficiency (insufficient suture) 22

Drug Treatments Stop causative/contributory medications Vitamin K or C Approach to Bleeding April 4, 1998 Drug Treatments Stop causative/contributory medications Vitamin K or C DDAVP epsilon amino caproic acid (Amicar) Topical procoagulants 23

Bone Marrow Diseases Acute leukemias Myelodysplasia Approach to Bleeding April 4, 1998 Bone Marrow Diseases Acute leukemias Myelodysplasia Myeloproliferative disorders P. vera dysfunctional platelets 24

Tests are normal-Now what? Approach to Bleeding April 4, 1998 Tests are normal-Now what? simple purpura senile purpura Factor XIII deficiency alpha-2-antiplasmin deficiency mild factor deficiency amyloidosis vascular disorders 25

Still more? Hereditary hemorrhagic telangiectasia scurvy Approach to Bleeding April 4, 1998 Still more? Hereditary hemorrhagic telangiectasia scurvy Ehlers-Danlos syndrome? Henoch-Schonlein purpura the un-diagnosable fibrinolytic defect 26

Summary History & Physical Examination Laboratory tests Approach to Bleeding April 4, 1998 Summary History & Physical Examination Laboratory tests screening tests specific diagnostic tests Diagnosis-specific therapy Factor replacement Drugs 27

Approach to Bleeding April 4, 1998 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

Approach to Bleeding April 4, 1998 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 30