Anstey A, Mayne K, Winter M, Van de Pette J, Pope F M
Department of Dermatology, Wycombe General Hospital, U.K.
Br J Dermatol. 1991 Aug;125(2):155-63. doi: 10.1111/j.1365-2133.1991.tb06063.x.
Fifty-one patients with Ehlers-Danlos syndrome were investigated for abnormalities of platelets and coagulation. Thirty-eight were examined prospectively and 13 retrospectively. A bleeding history was taken from all patients; only four (8%) gave no history of a bruising or bleeding tendency. Nine patients (18%) had significant haemostatic abnormalities of whom four (8%) had a platelet release defect, three (6%) had a factor XI deficiency and two (4%) had a factor XIII deficiency. Additionally 16 patients (31%) had mild abnormalities of uncertain significance of whom four (8%) had prolonged bleeding times (three in association with platelet aggregation abnormalities), 13 (26%) had platelet aggregation abnormalities and two had a positive Hess test. Twenty-four patients (47%) had normal tests for haemostasis of whom 20 (39%) had a bleeding diathesis and four (8%) had no such tendency. Results were analysed according to the type of Ehlers-Danlos syndrome, but there was no pattern to the abnormalities. The high frequency of a bleeding tendency in Ehlers-Danlos patients with normal tests for haemostasis (83%) supports the conventional explanation for this clinical feature, that defects in the structural integrity of skin and blood vessels lead to easy bruising.
对51例埃勒斯-当洛综合征患者的血小板和凝血功能异常情况进行了调查研究。其中38例为前瞻性研究对象，13例为回顾性研究对象。收集了所有患者的出血史；只有4例（8%）没有淤青或出血倾向的病史。9例患者（18%）存在明显的止血功能异常，其中4例（8%）有血小板释放缺陷，3例（6%）缺乏凝血因子XI，2例（4%）缺乏凝血因子XIII。此外，16例患者（31%）存在意义不确定的轻度异常，其中4例（8%）出血时间延长（3例伴有血小板聚集异常），13例（26%）有血小板聚集异常，2例赫斯试验呈阳性。24例患者（47%）的止血功能检查结果正常，其中20例（39%）有出血素质，4例（8%）没有这种倾向。根据埃勒斯-当洛综合征的类型对结果进行了分析，但异常情况并无规律可循。止血功能检查正常的埃勒斯-当洛综合征患者中出血倾向的高发生率（83%）支持了对这一临床特征的传统解释，即皮肤和血管结构完整性缺陷导致容易出现淤青。
