Glanzmann's thrombasthenia is an extremely rare, autosomal recessive disorder of the blood, in which the platelets lack GPIIb/IIIa. Hence, no fibrinogen bridging can occur, and bleeding time is significantly prolonged.

Clinical Features

Characteristically, there is increased mucosal bleeding:

• epistaxis
• menorrhagia
• increased bleeding post-operatively.

The bleeding tendency is variable but may be severe.

Patients present with moderate bleeding and normal platelet morphology. Aggregation of platelets occurs in response to ristocetin , but not to other agonists such as ADP, thrombin, collagen or adrenalin.

See also:

• Coagulation