von Willebrand Disease

Predisposing factors/ Epidemiology:

Most common inherited bleeding disorder. Autosomal dominant with varying expression.

Signs & Symptoms

Mucous membrane bleeding (e.g. epistaxis, menorrhagia), excessive blood loss from superficial cuts and abrasions, operative and post-traumatic hemorrhage.


Abnormal function or reduced level of vWF resulting from a point mutation or major deletion.

Peripheral blood characteristics

Lymphocytosis with small lymphocytes. Smudge or smear cells may be present. Normochormic normocytic anemia due to marrow infiltration or hypersplenism.

Important investigations & related results

Prolonged BT, and maybe APTT. Factor VIII and vWF levels are low. Platelet count is normal except in type 2B where it is low.


Type 1: Quantitative partial deficiency, Type 2: Functional abnormality, Type 3: Complete deficiency.

Treatment & management

Antifibrinolytic agent.

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