Primary T-cell lymphoma of the brain in a patient at advanced stage of acquired immunodeficiency syndrome

Abstract

Objective: To present, to our knowledge, the first case of primary central nervous system T-cell lymphoma in the setting of acquired immunodeficiency syndrome. Design.Clinical, radiological, laboratory, and pathological data from a young patient at advanced stage of acquired immunodeficiency syndrome were studied.

Results: A 38-year-old white man with stage CIII human immunodeficiency virus infection presented with headaches. Cerebrospinal fluid culture was positive for mycobacterium. He had facial herpes simplex virus and disseminated cytomegalovirus infections. Magnetic resonance imaging of the brain showed multiple areas of patchy or ring-shaped contrast enhancement in the periventricular zone of the right frontoparietal region. Stereotactic brain biopsy showed perivascular CD3 (pan T-cell marker)-positive lymphocytic infiltrates with partial necrosis extending into the neuropil. Not a single cell was labeled with L26, a B-cell marker. Negative and positive controls were appropriate. Special stains for cytomegalovirus, herpes simplex virus, Epstein-Barr virus, acid-fast bacilli, and fungi were negative. Gene rearrangement studies showed rearranged Tgamma and Tbeta bands. Polymerase chain reaction with a primer specific for Epstein-Barr virus genome-encoding nuclear antigen 1 region was positive. Human T-cell leukemia/lymphoma virus I work-up was negative. The patient was treated for mycobacteria. Four months later, he presented with a paravertebral neck mass. Needle biopsy showed a T-cell lymphoma with morphology similar to that in the brain.

Conclusions: To our knowledge, this case represents the first example of T-cell lymphoma of the brain in a patient with advanced stage of acquired immunodeficiency syndrome. Epstein-Barr virus may play a role in T-cell lymphomagenesis. The other unique finding is the complete absence of B cells in a central nervous system lesion. The underlying mechanism eradicating the B cells is unknown.