Agarwal M B, Agarwal U M, Viswanathan C, Bhave A A, Billa V
Department of Hematology, L.T.M.G. Hospital, Dadar, Bombay.
Indian Pediatr. 1992 Jul;29(7):837-41.
During January 1981 to June 1991, 20 patients from 16 unrelated families were detected to have Glanzmann's thrombasthenia (GT). Twelve families (75%) had history of consanguinity, with 6 first cousins and 3 uncle-niece marriages; of these 7 were Muslims, 6 Hindus and 3 Christians. There were 12 girls and 8 boys; the mean age at diagnosis was 7.05 +/- 6.03 yr (range 1 day-22 yr). All cases had initial bleeding prior to the age of 5 yr with the mean age at the initial episode of bleeding being 2.21 +/- 1.34 yr (range 1 day-5 yr). Common pattern of bleeding included epistaxis, gingival bleeding, post-traumatic bruises, menorrhagia, gastrointestinal (2 cases), post-operative (2 cases) and spontaneous bleeding (2 cases). No patient showed hemarthrosis, intracranial bleeding or hemoptysis. Menorrhagia was a serious problem necessitating repeated transfusions and hormonal therapy. Twelve cases (60%) required 1-120 units of blood transfusions while five received platelet concentrates.
在1981年1月至1991年6月期间，检测发现来自16个无亲缘关系家庭的20名患者患有Glanzmann血小板无力症（GT）。12个家庭（75%）有近亲结婚史，其中6对是表亲，3对是叔侄通婚；这些家庭中7个是穆斯林，6个是印度教徒，3个是基督教徒。有12名女孩和8名男孩；诊断时的平均年龄为7.05±6.03岁（范围1天至22岁）。所有病例在5岁之前均有初次出血，初次出血的平均年龄为2.21±1.34岁（范围1天至5岁）。常见的出血模式包括鼻出血、牙龈出血、创伤后瘀斑、月经过多、胃肠道出血（2例）、术后出血（2例）和自发性出血（2例）。没有患者出现关节积血、颅内出血或咯血。月经过多是一个严重问题，需要反复输血和激素治疗。12例（60%）需要输注1至120单位血液，5例接受了血小板浓缩物治疗。
