Chapin J E, Davis L E, Kornfeld M, Mandler R N
Department of Neurology, University of New Mexico School of Medicine, Albuquerque, USA.
Acta Neurol Scand. 1995 Jun;91(6):494-9. doi: 10.1111/j.1600-0404.1995.tb00452.x.
Intravascular lymphomatosis is a rare fatal neoplasm characterized by malignant cells of lymphocytic lineage producing vascular occlusions. The cerebral vasculature is particularly affected. Two patients seen at our institution presented with progressive neurologic deficits including dementia, hemiparesis and myelopathy. Review of an additional 64 reported cases with neurologic involvement indicates that patients developed intermittent fevers, an encephalopathy ranging from acute disorientation to rapidly progressive dementia, and focal signs such as hemiparesis and myelopathy. Common laboratory abnormalities include elevated cerebrospinal fluid protein and a lymphocytic pleocytosis, elevated blood erythrocyte sedimentation rate and serum lactate dehydrogenase. Malignant cells are rarely seen in cerebrospinal fluid, blood or bone marrow. Neuroimaging is usually abnormal with parenchymal lesions seen on cerebral tomography and magnetic resonance imaging along with an occasional meningeal pattern of contrast enhancement. Treatment with corticosteroids, chemotherapy, radiation therapy, or plasmapheresis provided limited benefit. Intravascular lymphomatosis should be considered in the differential diagnosis of unexplained progressive encephalopathy with superimposed focal deficits.
血管内淋巴瘤病是一种罕见的致命性肿瘤，其特征是淋巴细胞系的恶性细胞导致血管闭塞。脑循环系统尤其易受影响。我们机构接诊的两名患者表现为进行性神经功能缺损，包括痴呆、偏瘫和脊髓病。回顾另外64例有神经受累的报道病例表明，患者出现间歇性发热、从急性定向障碍到快速进展性痴呆的脑病，以及偏瘫和脊髓病等局灶性体征。常见的实验室异常包括脑脊液蛋白升高和淋巴细胞增多、血沉加快和血清乳酸脱氢酶升高。在脑脊液、血液或骨髓中很少见到恶性细胞。神经影像学检查通常异常，脑CT和磁共振成像可见实质病变，偶尔可见脑膜强化模式。使用皮质类固醇、化疗、放疗或血浆置换治疗效果有限。在鉴别诊断原因不明的进行性脑病合并局灶性缺损时应考虑血管内淋巴瘤病。
