Kara A, Yarali N, Fisgin T, Duru F
Department of Pediatric Hematology, Dr Sami Ulus Children 's Hospital, Ankara, Turkey.
Acta Paediatr. 2002;91(10):1139-40. doi: 10.1080/080352502760311683.
Glanzmann thrombasthenia is a rare hereditary qualitative platelet disorder characterized by a lifelong bleeding tendency due to quantitative and qualitative abnormalities of the platelet integrin alpha(IIb)beta3. Common clinical manifestations include purpuric type skin bleeding, prolonged bleeding from minor cuts, epistaxis, gingival bleeding and menorrhagia. Less frequently, gastrointestinal system bleeding may occur. Haemarthrosis, haematuria, intracranial and visceral haemorrhage are very rare symptoms. This study reports a 3-y-old girl with Glanzmann thrombasthenia who presented with life-threatening haemothorax. There was no history of recent trauma or drug usage and no vascular or parenchymal abnormalities to explain the development of haemothorax.
To the authors' knowledge this is the first case of Glanzmann thrombasthenia complicated by spontaneous haemothorax.
Glanzmann血小板无力症是一种罕见的遗传性血小板质量异常疾病，其特征是由于血小板整合素α(IIb)β3的数量和质量异常而导致终身出血倾向。常见临床表现包括紫癜型皮肤出血、小伤口出血时间延长、鼻出血、牙龈出血和月经过多。较少见的情况是，可能发生胃肠道系统出血。关节积血、血尿、颅内和内脏出血是非常罕见的症状。本研究报告了一名3岁患有Glanzmann血小板无力症的女孩，她出现了危及生命的血胸。近期没有外伤或用药史，也没有血管或实质异常来解释血胸的发生。
据作者所知，这是首例Glanzmann血小板无力症并发自发性血胸的病例。
