Management and Outcomes of HIV-Associated Primary Effusion Lymphoma: A Single Center Experience

Primary effusion lymphoma (PEL) is a rare form of lymphoma, primarily seen in individuals who are immunosuppressed. We studied all cases of PEL seen at our institution over a 15-year period and observed that if affected mostly male patients with HIV who were not taking the antiretroviral medication. Newer chemotherapy regimens including the drug bortezomib may be more effective in treating PEL. Background Primary effusion lymphoma (PEL) is a rare malignancy usually associated with HIV infection. Management and outcomes are poorly understood. Methods The medical records of all patients diagnosed with HIV-associated PEL at our institution between 1999 and 2014 were reviewed. Patients were followed till death, treatment failure or loss of follow-up. Results Twelve patients with PEL were identified during the 15 year study period; 9 had HIV infection. All 9 were male; median age was 45 years. All presented with local symptoms and were diagnosed with PEL a median of 11 years after HIV diagnosis. Location was pleural (3), pericardial (3), peritoneal (1) and extracavitatory (2). By definition, all had Ann Arbor stage 4 at diagnosis. Median follow-up was 34 months. Two patients had poor performance status and were unable to get chemotherapy. Seven patients had a complete remission (CR) and two died within 1 month of diagnosis. The median CD4 levels at PEL diagnosis in patients with poor versus good outcomes were 54 cells/mm3 (range, 26-82 cells/mm3) and 211 cells/mm3 (range, 73-800 cells/mm3). In contrast, the median lactate dehydrogenase (LDH) levels at PEL diagnosis with poor versus good prognosis were 1074 U/L (range, 703-1445 U/L) and 283 U/L (range, 156-760 U/L). Conclusions Given its rarity, our knowledge of PEL relies solely on case reports and case series. Prompt HAART and chemotherapy may be effective in HIV- associated PEL and good outcomes are possible. LDH and CD4 may be possible prognostic factors in PEL.