Bleeding can be severe with injury or surgery. Males and females are affected equally. Symptoms are consistent with low or dysfunctional platelets and include easy bruising, nosebleeds, mucosal bleeding, menorrhagia, and, occasionally, gastrointestinal bleeding. The severity of symptoms may be widely variable. The physical examination is consistent with low or dysfunctional platelets and may include increased bruising and mucosal bleeding.
Heterozygotes usually have no bleeding manifestations.
Giant platelets are seen on the peripheral smear. Bleeding time usually is prolonged. This is not corrected by the addition of normal plasma, as seen in von Willebrand disease. Flow cytometry can demonstrate abnormalities of platelet membrane glycoprotein. For surgery or life-threatening hemorrhage, platelet transfusion is the only available therapy.