Glanzmann’s Thrombasthenia

Objective: To look into the pattern of clinical presentation of patients of GT in our setup Material and Methods: A Retrospective study on patients with diagnosis of Glanzmann’s thrombasthenia was conducted at Hemophilia Treatment Centre, Pakistan Institute of Medical Sciences (PIMS). All the patients registered from January 2005 to December 2005 were included in the study. Their detailed clinical history specially age, sex age at diagnosis, family history, pattern of bleeding and treatment history were studied. All their investigations including platelet function studies were evaluated. Results: In our study platelet function defects were found in 8.9% of patients presenting with bleeding disorders, and amongst them Glanzmann’s thrombasthenia was observed in 89.6% cases. Consanguinity was observed in 86.5 of our patients. Bleeding from nose and gums were the commonest sites, however in women menorrhagia was the most frequent complaint. Antifibrinolytic agents were used in all cases, platelet transfusion in twenty two cases and Recombinant FVIIa was given two patients. Conclusion: Among platelet function defects, Glanzmann’s thrombasthenia was the most common (89.6%). Consanguinity was observed in 86.5% cases and 42.3% patients had the disease in other family members as well. Bleeding from nose and gums was the most common. HCV Positivity was seen in 11.5% patients