TY - JOUR
T1 - Primary human herpesvirus 8-negative effusion-based lymphoma
T2 - A large B-cell lymphoma with favorable prognosis
AU - Kaji, Daisuke
AU - Ota, Yasunori
AU - Sato, Yasuharu
AU - Nagafuji, Koji
AU - Ueda, Yasunori
AU - Okamoto, Masataka
AU - Terasaki, Yasushi
AU - Tsuyama, Naoko
AU - Matsue, Kosei
AU - Kinoshita, Tomohiro
AU - Yamamoto, Go
AU - Taniguchi, Shuichi
AU - Chiba, Shigeru
AU - Ohshima, Koichi
AU - Izutsu, Koji
N1 - Funding Information:
Conflict-of-interest disclosure: D.K. has received honoraria from Eisai, Chugai, Janssen, Sanofi, Kyowa Kirin, Celgene, Asahi KASEI Pharma, Mundipharma, and Mitsubishi Tanabe. M.O. has received scholarship donations from Chugai, Kyowa Kirin, Takeda, and Taiho. K.M. has received honoraria from Janssen, Takeda, and Celgene. G.Y. has received grants from Chugai and honoraria from Chugai, Mundipharma, Bristol Myers Squibb, Celgene, Ono, Daiichi-Sankyo, Eisai, Kyowa Kirin, and Takeda. S.T. has received grants from Chugai and Kyowa Kirin; and honoraria from Janssen, Asahi KASEI Pharma, Eisai, Otsuka Pharma, Novartis, Pfizer, MSD, Astellas Pharma, Mundipharma, Ono, Kyowa Kirin, Chugai, Celgene, Mochida Pharma, Bristol Myers Squibb, Dainihon Sumitomo, Fuji-moto Pharma, and Sanofi. S.C. has received honoraria from Thyas Co, Kyowa Kirin, Astellas Pharma, Ono, Sanofi, Chugai, and Takeda. K.I. has received grants from Chugai; and honoraria from Chugai, Celgene, Eisai, Novartis, Abbvie, Janssen, Kyowa Kirin, Takeda, MSD, AstraZeneca, FUJIFILM Toyama Chemical, Ono, Nihon Mediphysics, Dainihon Sumitomo, Bayer, HUYA Japan, Bristol Myers Squibb, Mundipharma, Otsuka, Daiichi Sankyo, Astellas, and Asahi KASEI Pharma. The remaining authors declare no competing financial interests.
Publisher Copyright:
© 2020 by The American Society of Hematology
PY - 2020/9
Y1 - 2020/9
N2 - 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.
AB - 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.
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UR - http://www.scopus.com/inward/citedby.url?scp=85092255880&partnerID=8YFLogxK
U2 - 10.1182/BLOODADVANCES.2020002293
DO - 10.1182/BLOODADVANCES.2020002293
M3 - Article
C2 - 32936906
AN - SCOPUS:85092255880
VL - 4
SP - 4442
EP - 4450
JO - Blood advances
JF - Blood advances
SN - 2473-9529
IS - 18
ER -
