Glass J, Hochberg F H, Miller D C
Department of Neurology, Temple University Health Sciences Center, Philadelphia, Pennsylvania 19140.
Cancer. 1993 May 15;71(10):3156-64. doi: 10.1002/1097-0142(19930515)71:10<3156::aid-cncr2820711043>3.0.co;2-o.
Intravascular lymphomatosis (IL) is a systemic neoplasm that often involves the nervous system, inducing progressive neurologic deficits in the setting of undiagnosed or quiescent extranodal non-Hodgkin lymphoma.
The clinical and pathologic files of the Massachusetts General Hospital and New York University Medical Center and the English language literature were reviewed to identify all reports of intravascular lymphomatosis (angioendotheliomatosis) or other examples of a diffuse proliferation of neoplastic cells filling capillaries, arterioles, and venules.
The authors report seven patients with IL and note 114 patients reported in the literature. Almost two-thirds (63%) of patients had neurologic manifestations, without abnormalities on bone marrow biopsy, chest and abdominal tomographic examinations for adenopathy, and cerebrospinal fluid (CSF) analysis. All patients had one or more of four syndromes, each reflecting a vascular occlusive process: progressive, multifocal cerebrovascular events; paraparesis, pain, and incontinence; a subacute encephalopathy; and peripheral or cranial neuropathies.
The unexplained presence of any one or more of these neurologic syndromes should alert the physician to the possible presence of this disease.
血管内淋巴瘤（IL）是一种全身性肿瘤，常累及神经系统，在未诊断或静止的结外非霍奇金淋巴瘤情况下导致进行性神经功能缺损。
回顾了麻省总医院和纽约大学医学中心的临床和病理档案以及英文文献，以确定所有血管内淋巴瘤（血管内皮瘤病）或其他肿瘤细胞弥漫性增殖填充毛细血管、小动脉和小静脉的病例报告。
作者报告了7例血管内淋巴瘤患者，并注意到文献中报道了114例患者。近三分之二（63%）的患者有神经学表现，骨髓活检、胸部和腹部淋巴结病的断层扫描检查以及脑脊液（CSF）分析均无异常。所有患者都有一种或多种四种综合征，每种综合征都反映了一种血管闭塞过程：进行性多灶性脑血管事件；双下肢轻瘫、疼痛和尿失禁；亚急性脑病；以及周围或颅神经病变。
这些神经综合征中任何一种或多种原因不明的出现应提醒医生注意这种疾病的可能存在。
