Glanzmann’s thrombasthenia in Iran

Background: Glanzmann’s thrombasthenia is a rare inherited disorder of platelet function in which platelets lack ability to bind fibrinogen and aggregate. The disease is characterized by glycoproteins IIb and/or IIIa deficiency. Objective: To investigate Glanzmanns thrombasthenia in Iran and evaluate the clinical signs and laboratory findings of patients. Methods: 342 patients with Glanzmann’s thrombasthenia who had been submitted by coagulation laboratory of Iranian blood transfusion service since 21 years ago were studied. The tests included platelet count, platelet morphology, bleeding time, clotting time, clot reaction, specific tests for PE-3 activity glass adhesion, platelet aggregation by aggregating agents. The information regarding all patients had been used. Findings: It was revealed that only 40% of patients were diagnosed before age of 5. The number of male patients were 50% more than females. Only 11% of patients had been diagnosed during one year after episode of mucosal bleeding. 90% of patients had familial history and mortalities due to bleeding. Conclusion: Regarding the high prevalence of familial marriage in Iran, Glanzmanns thrombasthenia is not considered as a rare disease. In case of sufficient lab facilities, the chance of patients diagnoses will become higher.