A RARE BLEEDING DISORDER: GLANZMANN‘S THROMBASTHENIA: A SERIES OF THREE CASES

Dr Purnima Kodate; Dr Fatema Topiwala; Dr D T Kumbhalkar; Dr W K Raut

Volume : VIII, Issue : IV, April - 2019

A RARE BLEEDING DISORDER: GLANZMANN‘S THROMBASTHENIA: A SERIES OF THREE CASES

Dr Purnima Kodate, Dr Fatema Topiwala, Dr D T Kumbhalkar, Dr W K Raut

Abstract :

Background: Glanzmann‘s thrombasthenia (GT), is a rare bleeding disorder which affects the megakaryocytic lineage. It is characterized by lack of platelet aggregation, due to quantitative and/or qualitative abnormalities of αIIb–β3 integrin, which is a receptor that helps in aggregation of platelets at the site of any endothelial injury. Cases: We present a series of 3 cases in young children. These cases were diagnosed on the basis of characteristic clinical features of Glanzmann thrombasthenia presenting with gingival bleed, petechial hemorrhage, epistaxis, injection site bleed. Laboratory parameters of prolonged bleeding time, abnormal clot retraction, absence of platelets clump on peripheral blood smear, platelet aggregometry and flow cytometry clinches the diagnosis. With accurate diagnosis and proper supportive care GT has a very good prognosis. Conclusion: Initial recognition and knowledge about etiopathogenesis may be of benefit to pathologists, haematologists and physicians who deal with hemostatic disorders to plan safe and effective care.