Primary Effusion Lymphoma Involving both Pleural and Abdominal Cavities in a Patient with Hepatitis B Virus-related Liver Cirrhosis

Abstract

Primary effusion lymphoma (PEL) is an unusual form of non-Hodgkin's lymphoma, which is characterized by lymphomatous effusion in body cavities, but no associated mass lesions. It is usually associated with an immunodeficient state most often with the human immunodeficiency virus (HIV). We describe a 54-year-old man with HIV-negative PEL, with a history of hepatitis B virus-related liver cirrhosis. Both abdominal and pleural cavities were involved; no solid tumor masses were found and bone marrow investigations were normal. The ascites and pleural effusion contained numerous pleomorphic lymphoid cells. Immunophenotyping was positive for CD138. Chromosome study showed complex cytogenetics. The genomic human herpesvirus-8 was detected in the lymphoma cells. It is postulated that the immunosuppressed state in this patient may have been caused by cirrhosis. The patient received four cycles of chemotherapy of CHOP and Picibanil (OK-432) intraperitoneal administration. However, no durable remission was achieved. Adefovir failed to halt the progressive liver failure after the development of YMDD mutant related to lamivudine. He died of sepsis and hepatic failure.

KHSV− EBV− post‐transplant effusion lymphoma with plasmablastic features: variant of primary effusion lymphoma?

“…Of interest, our case and a post-transplant PEL displayed aberrations of chromosome 19 [8], which have also been described in KSHV þ PEL [6]. Our case also displayed abnormalities of chromosomes 1, 3, 5 and 8, in common with previously published cases of post-transplant PELs [7][8][9] and KSHV À PEL-like lymphomas associated with chronic liver disease [15,18,19,21].…”

“…Around 30 cases of such lymphomas have been described, mostly in HIV-infected or immunocompetent adults [14]. However, a few cases of KSHV À PEL-like PTLDs have been published [22][23][24], and there is a growing literature of KSHV À PEL-like lymphomas occurring in patients with chronic liver disease, mostly due to HCV infection, [15][16][17][18][19][20][21] (Table 2).…”

“…Lymphomas resembling PEL, presenting as effusions without demonstrable mass lesions, have also been described. These include KSHV À EBV À effusion lymphomas occurring in HIV-infected or immunocompetent individuals [14] or in patients with chronic liver disease [15][16][17][18][19][20][21], as well as KSHV À EBV þ effusion post-transplant lymphoproliferative disorders (PTLDs) [22][23][24] (Table 2). We describe a KSHV À EBV À effusion lymphoma arising in an HIV À individual after combined liver and kidney transplantation, which displayed morphologic and phenotypic features of plasmablastic differentiation and was associated with serum paraproteins.…”

An unusual case of Primary Effusion Lymphoma with aberrant T-cell phenotype in a HIV-negative, HBV-positive, cirrhotic patient, and review of the literature

“…[1516] There are also published reports that describe PEL associated with liver cirrhosis and as a simultaneous infection with either Hepatitis B or C virus. [1718]…”

Report of an HIV and HHV-8 negative case of primary effusion lymphoma with idiopathic T4 lymphocytopenia

“…Subsequently, it was correlated with other immunodeficiency states, regardless of the presence of HHV-8/KSHV infection. PEL has been diagnosed in the setting of hepatitis-B virus (HBV)-related [8] or hepatitis-C virus (HCV)-related cirrhosis [9][10][11], increased age [12], solid organ transplantation [13] or common variable immunodeficiency [14]. However, a number of cases in previously immunocompetent individuals have also been reported [15][16][17].…”