PRIMARY CENTRAL NERVOUS SYSTEM LYMPHOMA AS FIRST MANIFESTATION OF AIDS. REPORT OF A CASE AND LITERATURE REVIEW

Abstract

Primary central nervous system lymphoma (PCNSL) is an infrequent neoplasm comprising only 5% of extranodal lymphomas. PCNSL is defined as a non-Hodgkin’s lymphoma (NHL) that is limited to the craniofacial axis without systemic involvement. This neoplasm is most common in patients with congenital and acquired immunodeficiencies in comparison with the general population. In AIDS patients, PCNSL occurs with a similar incidence in all the risks groups and ages with a high incidence in individuals with a count of less than 50 CD4 T cells/µL. This tumor is characteristically a large B-cell lymphoma strongly related to the presence of the Epstein Barr virus (EBV) genome in the atypical cells. The presence of EBV is detected in all AIDS-associated PCNSL but this finding is not observed in other patients with the same neoplasm. In AIDS patients with cerebral mass lesions, the detection of EBV in the cerebrospinal fluid (CSF) by DNA polymerase chain reaction (PCR) has a sensitivity of 83% to 100% and specificity higher than 90%. The use of highly active antiretroviral treatment (HAART) is associated with a decline in the incidence of AIDS-related PCNSL. The prognosis of AIDS-associated PCNSL is generally poor with a median survival of two to three months in the pre HAART era and a mild improvement in overall survival in the HAART era. Here we describe a patient who developed a PCNSL as a first manifestation of AIDS and we performed a review of the literature.