GLANZMANN'S THROMBASTHENIA - A CASE REPORT

Glanzmann's thrombasthenia is a very rare autosomal recessive platelet disorder due to mutation of gene for platelet aggregatory GPIIb-IIIa receptors. It affects megakaryocyte lineage and is marked by prolonged bleeding time, normal platelet count,lack of platelet aggregation in response to all except ristocetin. Patients usually manifest with mucocutaneous bleeds- epistaxis,gum bleeds, purpura, menorrhagia and rarely haematuria and gastrointestinal bleed. Here we report a 5 years old boy presented with recurrent epistaxis who was diagnosed as glanzmanns thrombasthenia.