A case of Glanzmann's thrombasthenia type I in a primigravida treated with factor VIIa

Glanzmann’s thrombasthenia is a rare autosomal recessive platelet function disorder. Though rare in the global context, this disorder is relatively more common in communities where consanguineous marriages are more frequent. The defect in platelet function is the inability of thrombasthenic platelets to aggregate. Their structural defect is the deficiency or dysfunction of platelet membrane (glycoprotein) GpIIb/IIIa complex which mediates platelet aggregation via fibrinogen binding. Epistaxis, gum bleeding, menorrhagia are the common clinical manifestations, whereas large muscle hematoma or hemarthrosis seldom occur in these patients. They often suffer from severe bleeding complications in case of injury or surgery. Pregnancy is uncommon and dangerous