Ai-Barghouthi S K, Ai-Othman A, Lardhi A
Department of Hematology, King Fahd Hospital of the University, Al-Khobar, Saudi Arabia.
J Family Community Med. 1997 Jan;4(1):57-61.
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.
The aim of the study is to find out the clinical presentation of Glanzmann's thrombosthenia in Saudi patients, Eastern Province.
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.
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.
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.
Glanzmann血小板无力症是一种罕见的遗传性出血性疾病，其特征为血小板功能异常。该病通常表现为皮下出血和黏膜出血。
本研究旨在了解沙特东部省份患者中Glanzmann血小板无力症的临床表现。
在本报告中，我们回顾了11年间在胡拜尔法赫德国王大学医院就诊的16例患有该疾病的沙特患者的临床表现。
我们注意到，除了我们的患者中关节积血明显更为常见（类似血友病）外，各种症状的发生频率与文献报道相似。
Glanzmann血小板无力症的临床症状范围似乎很广，各种症状的发生率各不相同。此外，一些表现特征往往类似于血友病，在某些情况下，这导致了使用含凝血因子VIII的血液成分进行不必要的治疗。
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