INTRAVASCULAR LYMPHOMATOSIS - A SYSTEMIC-DISEASE WITH NEUROLOGIC MANIFESTATIONS

Background. Intravascular lymphomatosis (IL) is a systemic neoplasm th at often involves the nervous system, inducing progressive neurologic deficits in the setting of undiagnosed or quiescent extranodal non-Hod gkin lymphoma. Methods. The clinical and pathologic files of the Massa chusetts General Hospital and New York University Medical Center and t he English language literature were reviewed to identify all reports o f intravascular lymphomatosis (angioendotheliomatosis) or other exampl es of a diffuse proliferation of neoplastic cells filling capillaries, arterioles, and venules. Results. The authors report seven patients w ith IL and note 114 patients reported in the literature. Almost two-th irds (63%) of patients had neurologic manifestations, without abnormal ities on bone marrow biopsy, chest and abdominal tomographic examinati ons for adenopathy, and cerebrospinal fluid (CSF) analysis. All patien ts had one or more of four syndromes, each reflecting a vascular occlu sive process: progressive, multifocal cerebrovascular events; parapare sis, pain, and incontinence; a subacute encephalopathy; and peripheral or cranial neuropathies. Conclusions. The unexplained presence of any one or more of these neurologic syndromes should alert the physician to the possible presence of this disease.