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Bleeding Disorders

Von Willebrand and Platelet Dysfunction
by

Lauren Gilliam

on 1 February 2013

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Transcript of Bleeding Disorders

von Willebrand and Factor Deficiencies Bleeding Disorders Approach in Primary Care Symptoms Easy bruising
Bleeding after trauma
Menorrhagia
Mucosal bleeding
Nose bleeds lasting >10m Diagnosis Presentation Biology History Physical Exam von Willebrand Initial tests:
von Willebrand factor antigen test
von Willebrand factor activity test (Ristocetin cofactor)
Factor VIII activity test Laboratory Tests Inspect for ecchymoses and hematomas
Current or recent mucosal bleeding
A negative physical examination is not uncommon. Lauren Gilliam
CHA/PA-S2 Recognize Risk Identify Symptoms Next Steps Management von Willebrand Mechanism of Disease Factor XII is not essential for normal coagulation, so it does not increase bleeding tendency in vivo. Clotting Factor Deficiencies References General and Systemic Pathology. 5th Edition. Underwood and Cross
Up to Date:
Clinical presentation and diagnosis of von Willebrand disease
Approach to the child with bleeding symptoms
Congenital and Acquired Platelet Disorders
HandwrittenTutorials.com Rare Recessive
Deficiency Rare Recessive
Deficiency "Common"
Deficiency Rare Recessive
Deficiency hemophilia A hemophilia B
"Christmas
disease" Common in
specific
populations. Rare Recessive
Deficiency Most Autosomal Dominant.
Type 1: quantitative deficiency
Type 2A & 2B: abnormal structure
Type 2M: abnormal binding to platelets
Type 2N: abnormal binding to factor VIII
Type 3: Homozygous (severe) Plasma concentration of vWF decreased, factor VIII activity decreased. Bleeding time prolonged as platelet interaction with subendothelium is slower. Carrier Protein
for VIII to last in
circulation by
increasing its
half-life to 12 hrs. Deficiencies in each of these factors impacts the coagulation cascade at different points, but all resulting in a slow response. No bleeding tendency (XII)
Subclinical bleeding tendency
Neonatal bleeding
(umbilical stump or intracranial bleed) Advise:
Do not take aspirin/NSAIDS
Prevent injuries when possible
Plan ahead for surgery or childbirth
Consider hormonal birth control 1% of the population, men and women equally. Nosebleeds
Gum bleeding
Bruising easily with lumps
Heavy or long-lasting periods
Bleeding more than expected
Bleeding when you took aspirin or NSAIDS Epistaxis lasting longer than 10 minutes
Lifelong easy bruising
Bleeding with or following dental extractions
Heavy menstrual bleeding
Bleeding during the peripartum period
Ingestion of aspirin/NSAIDS can precipitate bleeding
Some patients remain asymptomatic
Family member with diagnosed VWD Physical Exam
Initial Laboratory Testing
Referral
Treatment Bleeding Child Initial tests:
Platelet count
Peripheral blood smear
PT/INR ( II, VII, and X)
aPTT (XII, XI, IX, and VIII)
fibrinogen activity level Increase production of vWF
desmopressin (DDAVP®; Stimate®)
Replace vWF
Humate P® and Alphanate®.
Prevent clots from dissolving
(Amicar®) or (Cyklokapron®)
Full transcript