Intravascular lymphomatosis. A report of ten patients with central nervous system involvement and a review of the disease process.

ABSTRACT

The clinical, radiographic, and pathological findings in ten cases of intravascular lymphomatosis with central nervous system involvement seen at our institution over a 15-year period are presented. Nine patients presented with a subacute, progressive multifocal neurologic disorder. Most patients had fever, anemia, and elevation of the erythrocyte sedimentation rate. As the illness evolved, computerized tomography scanning and magnetic resonance imaging showed evidence of multifocal central nervous system disease. Angiography was nondiagnostic but suggested vasculitis in six cases. A response to empiric corticosteroid treatment was typical but usually transient. In six patients, the diagnosis was made antemortem by brain biopsy. The prognosis of patients was primarily dependent on early diagnosis and treatment, before massive central nervous system damage occurred. Treatment with chemotherapy, with or without radiotherapy, was associated with stabilization of the disease in three of five patients.