Plasmablastic Lymphoma in the Testis and Duodenum: A Case Report and Literature Review

Abstract

Plasmablastic lymphoma (PBL) is a rare and highly aggressive subtype of non-Hodgkin’s lymphoma (NHL) associated with human immunodeficiency virus (HIV) and Epstein-Barr virus (EBV). There are over 300 reported cases of PBL, of which over 120 are reported in HIV-positive patients. Of HIV-negative patients, patients were either immunocompetent or immunosuppressed transplant recipients. Initial reports highlighted cases arising in the oral cavity of HIV patients and this remains the most prevalent site of disease. However, other sites such as the gastrointestinal tract and skin are the next most common sites. Only three cases have involved the testes, a sanctuary site. Of gastrointestinal sites, there are only a handful of cases with gastric involvement. In the pre-human antiretroviral therapy (HAART) era, median overall survival was dismal. However, prognosis has improved since their advent. There is no significant difference in the survival of HIV-positive and HIV-negative PBL patients. It should be emphasized that due to the scarcity of cases, there are no established standards of care or prospective therapeutic trials for the management and treatment of PBL. Chemotherapy remains the standard approach, though selecting a regimen is still controversial and while response has been good, overall survival remains poor. Since 1995, autologous hematopoietic cell transplantation (AHCT) has been the standard of care for relapsed chemosensitive HIV-negative NHL. In the era of HAART, this has become feasible for HIV patients. A few studies have suggested that use of AHCT in the first line setting and relapsed or refractory disease may confer better outcomes. Herein we describe a unique case of involvement in the testis and duodenum, and there are no previous reports of a patient presenting with both gastrointestinal and urogenital involvement. J Hematol. 2015;4(3):196-201 doi: http://dx.doi.org/10.14740/jh217w