Primary human herpesvirus 8–negative effusion-based lymphoma: a large B-cell lymphoma with favorable prognosis

Abstract

Primary effusion-based lymphoma (EBL) presents as a malignant effusion in a body cavity. The clinicopathologic features and prognosis of primary human herpesvirus 8 (HHV8)–negative EBL remain unclear. We therefore conducted a retrospective study of 95 patients with EBL, regardless of HHV8 status, in Japan. Of 69 patients with EBL tested for HHV8, a total of 64 were negative. The median age of patients with primary HHV8-negative EBL at diagnosis was 77 years (range, 57-98 years); all 58 tested patients were negative for HIV. Primary HHV8-negative EBL was most commonly diagnosed in pleural effusion (77%). Expression of at least 1 pan B-cell antigen (CD19, CD20, or CD79a) was observed in all cases. According to the Hans algorithm, 30 of the 38 evaluated patients had nongerminal center B-cell (non-GCB) tumors. Epstein-Barr virus–encoded small RNA was positive in 6 of 45 patients. In 56 of 64 HHV8-negative patients, systemic therapy was initiated within 3 months after diagnosis. Cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) or CHOP-like regimens with or without rituximab (n = 48) were the most common primary treatments. The overall response and complete response rates were 95% and 73%, respectively. Three patients did not progress without systemic treatment for a median of 24 months. With a median 25-month follow-up, the 2-year overall survival and progression-free survival rates were 84.7% and 73.8%. Sixteen patients died; 12 were lymphoma-related deaths. Thus, most EBL cases in Japan are HHV8-negative and affect elderly patients. The non-GCB subtype is predominant. Overall, primary HHV8-negative EBL exhibits a favorable prognosis after anthracycline-based chemotherapy.

Primary age‐related EBV‐associated effusion‐based lymphoma successfully treated with rituximab and thoracentesis

“…9 Unlike PEL, which typically carries a poor prognosis due to resistance to chemotherapy, the estimated overall survival for patients with HHV8-negative EBL at 2 years following diagnosis has recently been reported to be as high as 84.7%. 3 Alexanian et al also reported that the overall response to chemotherapy in patients with HHV8-negative EBL was 82.1% compared with 39.6% in patients with PEL. 2 In another review, treatment with aspiration only or chemotherapy in HHV8negative EBL patients was far more efficacious than in PEL patients; complete or partial remission was attained by aspiration or chemotherapy in 70% and 82% of HHV8-negative EBL patients versus 18% and 39% of PEL patients, respectively.…”

“…HHV8‐negative EBL has been found in association with hepatitis C virus (HCV) infection, fluid overload, liver cirrhosis, renal dysfunction, cardiac arrhythmias, myocardial infarction, and heart failure 2‐5 . Epstein‐Barr virus (EBV) infection has been reported in 13% of HHV8‐negative EBL cases 3 . EBV‐positive and HHV8‐negative EBL have been reported in patients with liver disease, HIV, lymphocytopenia, common variable immunodeficiency, solid organ transplant, and renal disease 2,6‐10 …”

“…The hallmark of a HHV8‐negative EBL diagnosis is negativity for HHV8 infection, thereby distinguishing itself from primary effusion lymphoma (PEL) which requires the presence of HHV8 infection 1 . HHV8‐negative EBL has been found in association with hepatitis C virus (HCV) infection, fluid overload, liver cirrhosis, renal dysfunction, cardiac arrhythmias, myocardial infarction, and heart failure 2‐5 . Epstein‐Barr virus (EBV) infection has been reported in 13% of HHV8‐negative EBL cases 3 .…”

“…[2][3][4][5] Epstein-Barr virus (EBV) infection has been reported in 13% of HHV8-negative EBL cases. 3 EBV-positive and HHV8-negative EBL have been reported in patients with liver disease, HIV, lymphocytopenia, common variable immunodeficiency, solid organ transplant, and renal disease. 2,[6][7][8][9][10] EBV-positive DLBCL-NOS is a well-defined entity in the World Health Organization (WHO) classification of B-cell lymphomas.…”

HHV8-Negative Effusion-Based Large B Cell Lymphoma Arising in Chronic Myeloid Leukemia Patients under Dasatinib Treatment: A Report of Two Cases

“…Kaji, D. et al reported the results of a retrospective analysis of a series of HHV8-negative primary effusion-based lymphoma, demonstrating a favorable outcome with anthracycline based chemotherapy. This report, however, did not provide any information on the concurrent CML treatment [ 14 ].…”