A rare case of glanzmann thrombasthenia

Glanzmann’s thrombasthenia is a rare congenital bleeding disorder, in which there is impaired or absent clot retraction with defective platelet aggregation. Patients usually present with mucocutaneous bleeding and excessive bleeding associated with trauma and/or surgery. Mostly, the disease presents with mild hemorrhage but If bleeding is severe enough, it will be life-threatening. Thrombasthenic Platelets are normal in number and morphology. Patients have aprolonged bleeding time, with deficient platelet aggregation. Basic biochemical basis for GT is quantitative or qualitative defect of GPIIb-IIIa also known as integrin αIIbβ3. Platelet Transfusion may shorten the bleeding time for a brief period. In few GT patients hemorrhagic symptoms have been earnest for the necessity of bone marrow transplantation. With supportive care hemorrhage can be managed but the course tends to be highly variable.