Age and CD20 Expression Are Significant Prognostic Factors in Human Herpes Virus-8-negative Effusion-based Lymphoma

Abstract

Human herpes virus-8 (HHV-8)-negative effusion-based lymphoma (HHV-8-negative EBL) can be distinguished from primary effusion lymphoma based on clinical and pathologic findings. Although the morphology between the 2 is similar and they both originate from body cavities with serous effusions and are characterized by lack of tumor masses, HHV-8-negative EBL generally occurs in older patients, and has favorable response to therapy and better prognosis than primary effusion lymphoma. However, no systematic studies have investigated prognostic factors in patients with HHV-8-negative EBL. In this report, clinical and pathologic characteristics of 67 cases of HHV-8-negative EBL, including 2 of our own cases, were analyzed. Univariate analyses revealed older age (70 y and above), Japanese ethnicity, pericardial effusion, CD20 expression, and chemotherapy with rituximab were significantly favorable prognostic factors. Peritoneal effusion was identified as an unfavorable prognostic factor. In the multivariate analysis, age and CD20 expression were independent prognostic factors (P=0.013 and 0.003, respectively). A past history of induced fluid overload, hepatitis C viral infection, and peritoneal effusion were significantly correlated with patients aged below 70 years, while pericardial and pleural effusions were significantly correlated with patients aged 70 years and above. A comparison of cases with and without CD20 expression revealed that Japanese ethnicity and pericardial effusion were significantly correlated with CD20 expression, whereas a past history of induced fluid overload and peritoneal effusion were significantly correlated with the absence of CD20. We concluded that older age and CD20 expression are significant and favorable independent prognostic factors of HHV-8-negative EBL.

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

“…Moreover, CD20 expression is a biomarker for better overall survival in HHV8-negative EBL patients. 17 In the case of our patient, CD20 positivity provided us with a targetable and less toxic treatment option in comparison to standard chemotherapy. Furthermore, immunostaining for HHV8 and plasma cell marker CD138 were both negative, eliminating a possible PEL diagnosis.…”

“…As such, CD20‐targeted therapy using monoclonal antibodies such as rituximab can be employed in most cases of HHV8‐negative EBL. Moreover, CD20 expression is a biomarker for better overall survival in HHV8‐negative EBL patients 17 …”

Primary HHV-8 (-) Effusion-Based Non-Germinal Center B Cell Diffuse Large B Cell Lymphoma Successfully Treated with Standard Anthracycline-Based Chemoimmunotherapy

“…In the case of PEBL, it has been further reported that increased age and CD20 positivity appear to be favorable independent prognostic factors, both of which were present in our patient. 10 For this reason, and as we and others have suggested elsewhere, CD20 targeting therapy with rituximab in combination with thoracentesis of effusion fluid seems to be an acceptable alternative treatment option in CD20 positive patients who cannot withstand CHOP-like chemotherapy due to increased age or an inadequate performance functional status. 11 , 14 , 18 Our patient was able to successfully complete R-CHOP after initial treatment with rituximab monotherapy and rituximab-cyclophosphamide.…”

Development of a novel cell line‐derived xenograft model of primary herpesvirus 8‐unrelated effusion large B‐cell lymphoma and antitumor activity of birabresib in vitro and in vivo

“…Cases of HHV8‐negative primary lymphomatous effusions have been reported and these are referred to collectively as primary HHV8‐unrelated effusion large B‐cell lymphoma, primary HHV8‐negative effusion‐based lymphoma, or HHV8‐unrelated PEL‐like lymphoma. Intriguingly, patients of East Asian origin—especially Japanese—account for approximately 60% of cases of reported HHV8‐unrelated effusion large B‐cell lymphomas 6‐13 . HHV8‐unrelated effusion large B‐cell lymphoma is often seen in individuals with underlying adverse medical conditions, such as liver cirrhosis and congestive heart failure, which lead to fluid overload in body cavities 7,8 .…”

“…HHV8‐unrelated effusion large B‐cell lymphoma is often seen in individuals with underlying adverse medical conditions, such as liver cirrhosis and congestive heart failure, which lead to fluid overload in body cavities 7,8 . HHV8‐unrelated effusion large B‐cell lymphoma exhibits an overlapping clinical presentation with PEL, but with a distinct immunophenotype (usually positive for B‐cell‐associated antigens) and lack of association with HIV 6‐10 . Clinical outcomes of cases of HHV8‐unrelated effusion large B‐cell lymphoma are relatively favorable compared with cases of PEL, although some have an aggressive clinical course with poor prognosis 6,11‐13 .…”