原发性中枢神经系统血管内恶性淋巴瘤1例报告并文献复习

Primary Intravascular Malignant Lymphomatosis Angiotropic BCell Lymphomas in Central Nervous System:A Case Report and Literature Review

目的:探讨原发性中枢神经系统血管内恶性淋巴瘤(IM L)的诊治规律。方法:报告并结合文献复习,分析1例IM L临床表现、影像学特征和病理学特点。结果:本病呈亚急性病程,多发性缺血性梗塞病灶合并中心灶性出血,始终无强化。病理示广泛血管内恶性B淋巴瘤细胞大量增殖,导致血管堵塞,免疫组化LCA、CD 20阳性,CD 45RO阴性。结论:IM L是一种罕见的系统性播散性、高级别的非霍奇金淋巴瘤,临床表现及影像学征象缺乏特异性。其预后不良,平均生存期<1 a。

Objective：To review and explore the diagnosis and therapeusis of primary intravascular lymphomatosis of central nervous system. Methods：Clinical manifestations ,image and histopathology characteristics were analyzed and the related literatures were reviewed. Results ： This patient underwent a subacute course of encephalopathy. The images illustrated multifocal ischemic lesions with hemorrhagic central lesion,and without enhancement. The pathological findings were extensive intravascular proliferation of neoplastic lymphocytes B and consequent cerebrovascular occlusion. The immunohistochemistry findings ： LCA （ ＋ ）, CD20 （ ＋ ）, CD45RO （ - ）. Conclusions ： Intravascular malignant lymphomatosis is an exceptional and disseminated high ranking non-Hodgkin lymphoma without specific clinical manifestation and image charateristics,and its prognosis is unfavourable with the mean survival time less than one year.