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Von Willebrand Disease (vWD)


Step 1: View clinicals

A 2 year old boy presents with continued bleeding from his mouth, following a fall 5 hours ago. Close questioning of his mother reveals a history of somewhat prolonged bleeding from immunisation sites, and other sites of minor injury. No medical attention was sought. There is no history of lethargy, weakness, or pica. His drug and family histories are unremarkable.

Step 2: Order all relevant investigations

Full Blood Count

WBC: 7,900/mm3 (6,000 - 17,000) Hb: 12.3 g/dL (10.5 -13.5) Hct: 35.4% (33.0 - 39.0) Platelets: 368,000/mm3 (156,000 - 369,000)

Clotting Profile

PT: 11 sec (10-13) APTT: 37 sec (24-33)

Hemostasis Factor Assays

vWF Ag: 0.16 U/mL (0.78-1.53) Ristocetin cofactor activity assay: <0.10 U/mL (0.50-1.50)

Blood Film

The peripheral blood film (blood picture) appears completely normal.

Step 3: Select appropriate management

Tranexamic Acid
Iron + Folate
vWF Concentrates

Score: ★★☆