Differentials
Mild hemophilia A
SIGNS / SYMPTOMS
Bleeding symptoms may be similar, although patients with VWD tend to have more mucosal bleeding symptoms.
By family history, VWD is inherited in an autosomal pattern and hemophilia in an X-linked pattern.
However, because of the high mutation rate in hemophilia A and because of variable expressivity in VWD, a positive family history may be absent in both.
INVESTIGATIONS
Patients with hemophilia A have decreased factor VIII but normal von Willebrand factor antigen and activity. For VWD all 3 are decreased. Special binding studies or DNA analysis are required to distinguish type 2N VWD from hemophilia A.
Inherited platelet function disorder
SIGNS / SYMPTOMS
Both platelet function disorders and VWD have predominant mucosal bleeding symptoms.
Rare severe platelet function disorders such as Glanzmann thrombasthenia and Bernard Soulier syndrome are autosomal recessive in inheritance, but most inherited platelet function disorders are autosomal dominant.
INVESTIGATIONS
Platelet aggregometry is abnormal in inherited platelet disorders and normal in VWD.
In nontype 2B VWD there may be decreased sensitivity to ristocetin-induced platelet agglutination, but this is less sensitive for the diagnosis of VWD than the ristocetin cofactor assay.
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