A CASE REPORT ON GLANZMANN’S THROMBASTHENIA

Glanzmann's thrombasthenia (GT) is a rare autosomal recessive disorder in which the platelet glycoprotein IIb/IIIa (GP IIb/IIIa) complex is either deficient or, dysfunctional. The incidence is about 1 in 1,000,000. It is more common in populations where marriage between blood relatives is common. The signs of GT occur early in life and include easy bruising, epistaxis and prolonged bleeding from minor injuries. Epistaxis, menorrhagia, postpartum bleeding and surgical bleeding can be life-threatening. Here we are presenting a case of 4 year old female child with recurrent epistaxis and gingival bleeding for past one year. Diagnosis associates prolonged bleeding time with absent platelet aggregation in response to all physiological stimuli except ristocetin, with normal platelet count and morphology. Coagulation tests such as prothrombin time and partial thromboplastin time are normal. The cure for the disease does not exist; the only effective therapy consists of transfusions of fresh platelets or platelet concentrates. With proper supportive care Glanzmann's thrombasthenia has a very good prognosis. Keywords: Glanzmann's thrombasthenia, Platelet disorder, Epistaxis, Glycoprotein IIb/IIIa (GP IIb/IIIa) complex, gingival bleeding, Platelet aggregation.