Classic and extracavitary primary effusion lymphoma in 51 HIV‐infected patients from a single institution

Abstract

Human immunodeficiency virus (HIV)‐associated primary effusion lymphoma (PEL) is a rare B‐cell non‐Hodgkin lymphoma with poor prognosis. Lymphoma cells are always infected with human herpesvirus‐8 (HHV‐8) and in most cases coinfected with Epstein‐Barr virus. In classic presentation, PEL is characterized by body cavity effusions with or without mass lesions. A variant with only extracavitary localization has also been described. We report on a large single‐center series of patients with PEL in the era of combined antiretroviral therapy (cART). The main objective was to compare the characteristics and the outcome of patients with classic (n = 34) and extracavitary (n = 17) variant PEL. At PEL diagnosis, no major difference was observed between the two groups in terms of demographic and HIV characteristics. Extracavitary localizations were exclusively nodal in six patients and involved various organs in 11 patients. Another HHV‐8‐associated disease was observed in 31 patients, Kaposi sarcoma in 25, and multicentric Castleman disease in 18 patients, without difference between the two groups. Thirty‐two patients were treated with CHOP associated with high‐dose methotrexate, 13 were treated with CHOP‐derived regimen alone, and six patients received low‐dose/no chemotherapy. Complete remission was achieved in 21 (62%) and seven (41%) patients of the classic and extracavitary groups, respectively. The median overall survival (OS) was 10.2 months. Despite a higher disease‐free survival in the extracavitary group, there was no difference in OS between the two variants. Based on this series, characteristics of classic and extracavitary variants were very close. Although prognosis of PEL remains very severe in cART era, the median survival compares favorably with earlier series. Am. J. Hematol. 91:233–237, 2016. © 2015 Wiley Periodicals, Inc.

HIV-Associated Malignant Lymphoma

“…This very rare entity is associated with a poor prognosis, even in the cART era, with a median OS in the range of 6-10 months [37,38]. No randomized trials exist.…”

Oncolytic Reactivation of KSHV as a Therapeutic Approach for Primary Effusion Lymphoma

“…This is conceptually similar to the mechanism of an oncolytic viruses-based therapy like the FDA-approved Talimogene Laherparepvec, which is designed to: (1) replicate specifically in cancer cells, but not in normal cells, (2) stimulate the host immune system to the tumor microenvironment, and (3) have their replication manageable by replication-suppressive drugs for patient safety. Accordingly, lytic reactivation of KSHV from latently infected cancer cells would theoretically be beneficial by providing additional highly specific direct tumor lytic effects as well as by evoking an increased cytotoxic immune response against cancer cells expressing lytic viral antigens.…”

“…The aggressive nature of PEL is evident by the poor median overall survival for this cancer, 10.2 months in one recent study in which patients received a multidrug cytotoxic regimen (3). Given the limited treatment options for this Non-Hodgkin’s lymphoma, novel, rationally designed therapeutic approaches are urgently needed.…”

Lymphoproliferative Disorders of the Lung

“…The number and type of involved cavities could be associated with median survival [178] . However, a retrospective single-center study did not find any statistical difference between patients with involvement of only one cavity and those with involvement of more than one cavity [165] . LDH level and CD4 positivity may be prognostic factors [179] .…”

“…PEL is distinguished from solid lymphoma with secondary effusion localization [109,117] . However, PEL has also been described in a solid form -called extracavitary PEL -that may occur as a relapse of classic PEL or as an initial presentation [164,165] . PEL is a rare lymphoma corresponding to 0.5% of all lymphoma cases and 1-8% of HIV-associated non-Hodgkin lymphoma cases [165][166][167] .…”