Transcript approach to bleeding disorders

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
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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
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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
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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
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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
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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
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Decreased synthesis of factors
Synthesis of abnormal factors
Increased fibrinolysis
Thrombocytopenia
Liver Disease
• Fresh frozen plasma
– replete factors
– WILL 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
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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?
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simple purpura
senile purpura
Factor XIII deficiency
alpha-2-antiplasmin deficiency
mild factor deficiency
amyloidosis
vascular disorders
Still more?
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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