PRIMARY CENTRAL-NERVOUS-SYSTEM LYMPHOMA

Objective: To compare the pathogenesis, clinical presentation, therapy, and prognosis of primary central nervous system lymphoma in immunocompetent persons with these characteristics of the disease in patients with AIDS. Data Sources and Extraction: All English-language papers published between 1980 and 1992 dealing with either lymphoma and the central nervous system or AIDS were reviewed. Patient characteristics, clinical presentation, histologic findings, and treatment and survival data were extracted from each case report and review. Data Synthesis: Data were available on 792 patients (from 40 reported series) with non-AIDS-associated primary central nervous system lymphoma and 315 patients (from 32 series) with AIDS-associated primary central nervous system lymphoma. Patients with AIDS initially consulted a physician more often when they had global neurologic symptoms compared with patients without AIDS, with more than 50% of the lesions on computed tomographic (CT) scans in patients with AIDS being ring-enhancing and multifocal, a pattern rarely described in immunocompetent patients. The overall survival of the patients without AIDS was 18.9 months compared with 2.6 months for patients with AIDS, with substantial differences remaining even for subgroups of patients similarly treated with radiation and chemotherapy. Conclusion: Primary central nervous system lymphoma is probably a substantially different disease in persons with and without AIDS with regard to patient characteristics, clinical and radiographic presentation, and prognosis. Recent advances in the treatment of this disease in patients without AIDS have not largely affected patients with AIDS. Substantial improvements in survival in these patients await advances in controlling their human immunodeficiency virus-associated disease.