Approach to Bleeding Disorders

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Approach to Bleeding Disorders

• Anne T. Neff, M.D.

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Evaluation of the patient

• History
• Physical Examination
• Laboratory Evaluation

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History

• Are you a bleeder?
• surgical challenges
• accidents injuries
• dental extractions
• menstrual history

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Type of Bleeding

• ecchymoses
• petechiae
• epistaxis
• deep soft tissue bleed
• hemarthroses
• GI bleeding

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Does it sound genetic?

• duration of bleeding history
• congenital v. acquired
• family history
• examine pedigree
• determine inheritance

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Medical History

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

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Physical Examination

• current hemorrhage
• nature and extent
• intercurrent illnesses
• liver disease
• petechiae/ecchymoses

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Laboratory Assessment

• Guided by history
• Screening tests
• PT
• aPTT
• platelet count
• fibrinogen
• thrombin time

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Specific Laboratory Tests

• Mixing studies
• patient and PNP mixed 11
• incubated 2 hours at 37o C
• perform clotting assay as usual
• Uncorrected - circulating anticoagulant
• Corrected - factor deficiency

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Circulating Anticoagulant

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

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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 Willebrands disease
• most common genetic bleeding disorder
• many different types

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von Willebrands Disease

• autosomal dominant except Type III
• patients range from asymptomatic to spontaneous
  bleeding similar to a severe hemophiliac
• characterized by mucocutaneous bleeding

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von Willebrands Testing

• aPTT
• Factor VIII activity
• von Willebrands Factor
• Ristocetin Cofactor
• von Willebrands Factor multimers

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von Willebrands 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

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von Willebrands 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)

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Other Congenital Defects

• Other Factor deficiencies
• Platelet defects
• very rare
• platelet aggregation studies
• electron microscopy
• bleeding time

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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

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Liver Disease

• Decreased synthesis of factors
• Synthesis of abnormal factors
• Increased fibrinolysis
• Thrombocytopenia

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Liver Disease

• Fresh frozen plasma
• replete factors
• WILL NOT CORRECT THE PT
• Cryoprecipitate
• fibrinogen
• Platelets

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Disseminated Intravascular Coagulation

• Treat the underlying cause

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Disseminated Intravascular Coagulation

• Replete deficient factors
• FFP
• cryoprecipitate
• platelets
• Role of heparin?

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Dont Forget!

• Factor XIV deficiency
• (insufficient suture)

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Drug Treatments

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

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Bone Marrow Diseases

• Acute leukemias
• Myelodysplasia
• Myeloproliferative disorders
• P. vera
• dysfunctional platelets

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Tests are normal-Now what?

• simple purpura
• senile purpura
• Factor XIII deficiency
• alpha-2-antiplasmin deficiency
• mild factor deficiency
• amyloidosis
• vascular disorders

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Still more?

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

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Summary

• History Physical Examination
• Laboratory tests
• screening tests
• specific diagnostic tests
• Diagnosis-specific therapy
• Factor replacement
• Drugs

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Question 1

• The patient with normal laboratories, dry IV
  sites, and blood gushing out a surgical drain
  probably has
• a. von Willebrands disease
• b. undiagnosed hemophilia
• c. mechanical bleeding
• d. a bad attitude

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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