Multiple Remissions of Extracavitary Primary Effusion Lymphoma Treated With a Single Cycle of Liposomal Doxorubicin in a Patient Infected With HIV

Abstract

Primary effusion lymphoma (PEL) is a rare human herpesvirus 8 (HHV8)–related large B cell lymphoma with plasmablastic, immunoblastic, or anaplastic features that often carries a poor prognosis. This lymphoma occurs mainly in patients with hiv infection, most often with Epstein–Barr virus (EBV) co-infection, and usually presents as body cavity effusions or, less commonly, as extracavitary lesions without effusion (EC-PEL). Chemotherapeutic treatment options are limited and require concurrent antiretroviral therapy (ART). Here, we report the case of an adult patient with HIV infection and chronic hepatitis E virus (HEV) co-infection who had low CD4 T cell recovery after years of ART. The patient then developed a cutaneous EC-PEL which rapidly regressed after 1 cycle of liposomal doxorubicin (LD) for his Kaposi sarcoma (KS) before treatment with chop chemotherapy. He had previously received numerous cycles of LD for cutaneous ks over 2 years. Because of the patient’s low CD4 T cell count, HEV co-infection, and earlier unexpected remission of EC-PEL before CHOP, the patient opted for a single trial of LD before other options. Surprisingly, he experienced a complete remission lasting 18 months. Subsequently, his EC-PEL relapsed twice at 31 and at 41 months after the initial diagnosis. Upon recurrence, a similar single cycle of LD was given, which again induced remission. The patient today is in complete remission after a total of 4 LD infusions over 54 months. This patient represents a unique case of HIV-with-HHV8–related, EBV-negative EC-PEL with chronic HEV coinfection, in which rapid remission was achieved after a single cycle of LD, suggesting an antiviral response in addition to the chemotherapeutic effect.