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 PTWILL 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