Diagnostic tool for Glanzmann's thrombasthenia clinicopathologic spectrum

Objective: To platelet aggregometry and describe the clinical spectrum of Glanzmann's thrombasthenia diagnosed by platelet aggregometry. Study Design: A case-series. Place and Duration of Study: This study was carried out at the clinical laboratories at the Aga Khan University Hospital, Karachi from January 2003 to January 2006. Patients and Methods: All patients irrespective of age and gender presenting with bleeding symptoms and having normal platelet count were evaluated. Demographic details, relevant clinical history along with results of complete blood count, bleeding time and platelet aggregation studies were retrieved through computerized data base and evaluated for the diagnosis of Glanzmann's thrombasthenia. Results: During the study period, 50 out of 2317 patients (2.2%) were diagnosed as Glanzmann's thrombasthenia by platelet aggregometry with male to female ratio of 0.85:1 and median age of 10.2 years (ranging from 3 months to 27 years). Common symptoms were epistaxis, oral and gingival bleed, bleeding from minor cuts and trauma that were observed in 46% of the patients; while 18%, 8% and 10% of them also complained of bruising, hematuria and bleeding per rectum respectively. Majority i.e. 86% had a bleeding time greater than 10 minutes. All patients had received blood or blood products for their bleeding episodes. Conclusion: Platelet aggregometry is a useful diagnostic modality for the assessment of Glanzmann's thrombasthenia. The disorder presents with muco-cutanoeus bleeding and was found to be a common cause of bleeding in our setup.