HHV8-Negative Primary Effusion Lymphoma of B-Cell Lineage: Two Cases and a Comprehensive Review of the Literature

Abstract

Primary effusion lymphoma (PEL) is a rare extranodal lymphoma that typically presents in a body cavity in the absence of a detectable tumor mass and that occurs predominantly in immunosuppressed individuals. The neoplastic lymphoid cells are frequently infected with human herpes virus 8 (HHV8), also known as Kaposi sarcoma herpes virus (KSHV). We describe two HIV-negative patients who presented with primary effusion lymphoma of B-cell lineage involving the pleural cavity, but whose tumor cells lacked infection by HHV8. We review the English language literature of HHV8-negative PEL of B-cell lineage and compare these lymphomas to HHV8-associated PEL with regard to clinical and pathological characteristics, therapy, and outcome.

Primary Body Cavity-Based Large B-Cell Lymphoma in an HIV and HHV-8 Negative, HCV Positive Patient: A Case Report and Literature Review

“…While the prognosis of HHV-8 positive is extremely unfavorable with a median survival after diagnosis of 2 to 3 months (without treatment) or 6 months (with treatment), HHV-8 negative PEL seems to have a better clinical outlook (4 months without treatment or 10 months with treatment). 19,20 Given a similar immunoprofile (CD19+; CD138, HHV-8, and EBV), morphology (large cell size), and clinical presentation, the current case fits well into this unique group of body cavity-based large B-cell lymphomas. 25,26 Recent studies suggest that in addition to invading hepatocytes, HCV can also infect B-lymphocytes, T-lymphocytes, and natural killer cells and this may serve as a direct way for HCV to induce neoplastic transformation.…”

“…[29][30][31][32][33][34][35] Data on the prognosis of HCV positive, HHV-8 negative primary body cavity-based large B-cell lymphoma is limited: the median survival of the 12 reported cases (including both treated and untreated) is 5 months. 19,20 Advanced liver disease and limited chemotherapy options are likely contributers to the observed short survival.…”

A Man With Pleural Effusion and Ascites

“…9 Pleural and peritoneal fl uid in the present patient showed large mature B cells that bore similar morphology to diff use large B-cell lymphoma and expressed CD20 and CD79A without CD138.…”

“…8 In contrast, patients with HIV/HHV8-negative PEL are older (74 years) compared with those with HIV/HHV8-positive PEL (44 years). 9 In addition, patients with HHV8-negative PEL tend to show a better survival median of 11 months compared with 4 to 6 months for patients with HIV/HHV8-positive PEL. 5…”

“…9 Novel approaches such as antiviral therapy and targeted cellular therapies are still under clinical evaluation. 4…”