Noda M, Kitagawa M, Tomoda F, Iida H
Department of Pathology, Toyama Medical and Pharmaceutical University, Faculty of Medicine, Japan.
Am J Clin Pathol. 1990 Aug;94(2):217-21. doi: 10.1093/ajcp/94.2.217.
A 62-year-old woman was admitted for evaluation of muscular weakness, skin pigmentation, dry mouth, and interstitial pneumonia. During the course of her stay, adult respiratory distress syndrome, hemolytic anemia, renal failure, neurologic dysfunction, and thrombocytopenia appeared. A clinical diagnosis of thrombotic thrombocytopenic purpura (TTP) accompanied by polymyositis and Sjögren's syndrome was made. She died two weeks after the beginning of plasmapheresis, and an autopsy was performed. Immunohistochemistry disclosed deposits of IgM, fibrinogen, and C1q in glomeruli and arterioles and deposits of C3 in small arteries. von Willebrand's factor antigen, which promotes the adhesion of platelets to the subendothelium, was positive in onion-peeled arteries of the kidney and the spleen. These results suggest that immune complexes may have triggered a sequence of events from vascular endothelial injury to TTP.
一名62岁女性因肌肉无力、皮肤色素沉着、口干和间质性肺炎入院评估。在住院期间，出现了成人呼吸窘迫综合征、溶血性贫血、肾衰竭、神经功能障碍和血小板减少症。临床诊断为血栓性血小板减少性紫癜（TTP）伴多发性肌炎和干燥综合征。她在血浆置换开始两周后死亡，并进行了尸检。免疫组织化学显示肾小球和小动脉中有IgM、纤维蛋白原和C1q沉积，小动脉中有C3沉积。促进血小板与内皮下粘附的血管性血友病因子抗原在肾和脾的洋葱皮样动脉中呈阳性。这些结果表明免疫复合物可能引发了从血管内皮损伤到TTP的一系列事件。
