Krieger C, Robitaille Y, Jothy S, Elleker G
Ann Neurol. 1982 Nov;12(5):489-92. doi: 10.1002/ana.410120515.
A 53-year-old man presented with a lumbosacral polyradiculoneuropathy and developed fluctuating encephalopathy suggestive of multifocal small vessel disease. Postmortem examination demonstrated multifocal vascular occlusion by undifferentiated cells confined to the intravascular space. Extravascular spread was found only in spleen and liver. The presence of lysozyme and absence of factor VIII in the cytoplasm of the malignant cells confirmed their histiocytic nature. This patient had an unusual intravascular form of malignant histiocytosis that must be included in the differential diagnosis of multifocal vascular disease.
一名53岁男性患者出现腰骶部多神经根神经病，并发展为提示多灶性小血管疾病的波动性脑病。尸检显示未分化细胞局限于血管腔内导致多灶性血管闭塞。仅在脾脏和肝脏发现血管外扩散。恶性细胞胞质中溶菌酶的存在和因子VIII的缺失证实了它们的组织细胞性质。该患者患有一种不寻常的血管内型恶性组织细胞增多症，在多灶性血管疾病的鉴别诊断中必须考虑到这一点。
