ترومبو آستني گلانزمن در ايران

Glanzmannʹs thrombasthenia in Iran

Background : Glanzmannʹs 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 I or IIIa deficiency. Objective : To investigate Glanzmanns thrombasthenia in Iran and evaluate the clinical signs and laboratory findings of patients. Methods : 342 patients with Glanzmannʹs 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 adhession , 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 thrombasthemia isnot considered as a rare disease. In case of sufficient lab facilities , the chance of patient diagnoses will become higher.