Udvardy M, Boda Z, Hársfalvi J, Pfliegler G, Rák K
Orv Hetil. 1989 Aug 13;130(33):1751-9.
Thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome have quite uniform or identical histopathological features and share very similar clinical characteristics, so thrombotic microangiopathy may be used as a common descriptive term of the two syndromes. During the past one and a half year five patients with thrombotic microangiopathy, receiving plasma therapy were observed by the authors. Owing to the early, correct diagnosis and plasma administration four patients had been recovered from the disease. The evaluation of clinical signs and symptoms, altogether with some simple laboratory data (megakaryocytic thrombocytopenia, nonimmune acquired haemolytic anaemia, renal insufficiency, neurologic alterations, etc.) remained essential in the recognition and treatment of thrombotic microangiopathy. After giving a short review of the literature the authors deal with the results of some more specific examinations (prostanoids, beta-thromboglobulin, the quantitative and qualitative analysis of Willebrand factor, etc.) which may give further data to the complex aetiopathogenesis of thrombotic microangiopathy.
血栓性血小板减少性紫癜和溶血性尿毒综合征具有相当一致或相同的组织病理学特征,且临床特征非常相似,因此血栓性微血管病可作为这两种综合征的通用描述性术语。在过去一年半的时间里,作者观察了5例接受血浆治疗的血栓性微血管病患者。由于早期诊断正确并给予了血浆治疗,4例患者已康复。临床症状和体征的评估,以及一些简单的实验室数据(巨核细胞性血小板减少、非免疫性获得性溶血性贫血、肾功能不全、神经改变等)在血栓性微血管病的识别和治疗中仍然至关重要。在对文献进行简要回顾后,作者探讨了一些更具特异性检查(前列腺素、β-血小板球蛋白、血管性血友病因子的定量和定性分析等)的结果,这些检查可能为血栓性微血管病复杂的病因发病机制提供更多数据。