Glanzmann's thrombasthenia
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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.