{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,5]],"date-time":"2026-11-05T00:08:45Z","timestamp":1793837325400,"version":"4.6.1"},"reference-count":72,"publisher":"American Society of Hematology","issue":"18","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2020,9,22]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>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)\u2013negative 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\u2013encoded 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.<\/jats:p>","DOI":"10.1182\/bloodadvances.2020002293","type":"journal-article","created":{"date-parts":[[2020,9,16]],"date-time":"2020-09-16T17:36:03Z","timestamp":1600277763000},"page":"4442-4450","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":51,"title":["Primary human herpesvirus 8\u2013negative effusion-based lymphoma: a large B-cell lymphoma with favorable prognosis"],"prefix":"10.1182","volume":"4","author":[{"given":"Daisuke","family":"Kaji","sequence":"first","affiliation":[{"name":"Department of Hematology, Toranomon Hospital, Tokyo, Japan;"},{"name":"Department of Hematology, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Ibaraki, Japan;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasunori","family":"Ota","sequence":"additional","affiliation":[{"name":"Department of Pathology, Research Hospital, Institute of Medical Science, University of Tokyo, Tokyo, Japan;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuharu","family":"Sato","sequence":"additional","affiliation":[{"name":"Department of Pathophysiology, Okayama University Graduate School of Health Sciences, Okayama, Japan;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koji","family":"Nagafuji","sequence":"additional","affiliation":[{"name":"Division of Hematology and Oncology, Department of Medicine, Kurume University School of Medicine, Kurume, Japan;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasunori","family":"Ueda","sequence":"additional","affiliation":[{"name":"Department of Hematology\/Oncology, Kurashiki Central Hospital, Kurashiki, Japan;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masataka","family":"Okamoto","sequence":"additional","affiliation":[{"name":"Department of Hematology, Fujita Health University School of Medicine, Toyoake, Japan;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasushi","family":"Terasaki","sequence":"additional","affiliation":[{"name":"Division of Hematology, Toyama City Hospital, Toyama, Japan;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8718-4599","authenticated-orcid":false,"given":"Naoko","family":"Tsuyama","sequence":"additional","affiliation":[{"name":"Department of Pathology, Tokyo Metropolitan Geriatric Hospital, Tokyo, Japan;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kosei","family":"Matsue","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology, Department of Medicine, Kameda General Hospital, Chiba, Japan;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomohiro","family":"Kinoshita","sequence":"additional","affiliation":[{"name":"Department of Hematology and Cell Therapy, Aichi Cancer Center, Nagoya, Japan;"},{"name":"Japanese Red Cross Aichi Blood Center, Seto, Japan;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Go","family":"Yamamoto","sequence":"additional","affiliation":[{"name":"Department of Hematology, Toranomon Hospital, Tokyo, Japan;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shuichi","family":"Taniguchi","sequence":"additional","affiliation":[{"name":"Department of Hematology, Toranomon Hospital, Tokyo, Japan;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shigeru","family":"Chiba","sequence":"additional","affiliation":[{"name":"Department of Hematology, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Ibaraki, Japan;"},{"name":"Department of Hematology, Faculty of Medicine, University of Tsukuba, Ibaraki, Japan;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koichi","family":"Ohshima","sequence":"additional","affiliation":[{"name":"Department of Pathology, Kurume 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