GLANZMANN's THROMBASTHENIA-SPECTRUM OF CLINICAL PRESENTATION ON SAUDI PATIENTS IN THE EASTERN PROVINCE

INTRODUCTION: Glanzmann's thrombasthenia is a rare inherited hemorrhagic disorder characterized by abnormal platelet function. It usually presents with subcutaneous bleeding and bleeding from the mucous membranes. OBJECTIVES: The aim of the study is to find out the clinical presentation of Glanzmann's thrombosthenia in Saudi patients, Eastern Province. METHODS: In this report we have reviewed the clinical presentation of sixteen Saudi patients suffering from this disorder presented at King Fahd Hospital of the University in AI-Khobar over a period of eleven years. RESULTS: We have noticed similarity in the frequency of the various symptoms with those reported in the literature except for the apparently more frequent hemarthrosis in our patients which mimics hemophilia. CONCLUSION: The spectrum of clinical presentations with Glanzmann's thrombosthenia appears to be wide and the occurrence of the various symptoms varies. Moreover, some of the presenting features tend to mimic those of hemophilia, a situation which, in some cases, has resulted in unnecessary treatment with Factor VIII-containing blood components.