Glanzmann's thrombasthenia- report of a case and review of the literature

Glanzmann thrombasthenia is an autosomal recessive bleeding disorder associated with mutation of the gene for GPIIbIIIa receptor of the platelets characterized by increased bleeding time, normal coagulation profile, normal platelet count and morphology, with an abnormal platelet functional assay. Although very rare worldwide, 1 in a million incidence, it is relatively common in our South Indian population due to high degree of consanguineous marriages. Patients usually present with mucocutaneous bleeding and excessive bleeding associated with trauma or surgery, usually nonspontaneous bleeds. Bleeding is managed by platelet transfusions. Bone marrow transplants have been used successfully in rare cases. With proper supportive care Glanzmann's thrombasthenia has a very good prognosis. We present this rare yet a relatively common case of our locality with review of the literature