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

Daisuke Kaji, Yasunori Ota, Yasuharu Sato, Koji Nagafuji, Yasunori Ueda, Masataka Okamoto, Yasushi Terasaki, Naoko Tsuyama, Kosei Matsue, Tomohiro Kinoshita, Go Yamamoto, Shuichi Taniguchi, Shigeru Chiba, Koichi Ohshima, Koji Izutsu

Research output: Contribution to journalArticlepeer-review

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 5 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.

Original languageEnglish
Pages (from-to)4442-4450
Number of pages9
JournalBlood Advances
Volume4
Issue number18
DOIs
Publication statusPublished - 09-2020

All Science Journal Classification (ASJC) codes

  • Hematology

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