{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T20:24:58Z","timestamp":1787775898002,"version":"build-2784847793"},"reference-count":43,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2025,1,15]],"date-time":"2025-01-15T00:00:00Z","timestamp":1736899200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["Z01\u2010BC010888"],"award-info":[{"award-number":["Z01\u2010BC010888"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2025,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:p>Primary effusion lymphoma (PEL) is an HIV\u2010associated B\u2010cell non\u2010Hodgkin lymphoma (NHL) caused by Kaposi sarcoma herpesvirus (KSHV). There is no validated prognostic model in PEL, and prognosis is thought to be poor compared to other HIV\u2010associated NHL. We derived the PEL\u2010Prognostic score (PEL\u2010PS) from an international real\u2010world training set of 59 patients with HIV\u2010associated PEL who received first\u2010line anthracycline\u2010containing chemotherapy from the HIV and AIDS Malignancy Branch at the National Cancer Institute (NCI) in the United States and the National Center for HIV Malignancy at the Chelsea and Westminster Hospital (CWH) in England from 2000 to 2022. We identified prognostic factors associated with overall survival (OS). In a multivariable Cox model, ECOG \u2265\u20093 (<jats:italic>p<\/jats:italic>\u2009=\u20090.007; hazard ratio [HR]\u2009=\u20094.0 [95% CI: 1.5\u201311.1]) and hemoglobin &lt;\u20098\u2009g\/dL (<jats:italic>p<\/jats:italic>\u2009=\u20090.006; HR\u2009=\u20093.8 [95% CI: 1.5\u20139.7]) were jointly associated with lower survival probability. The resulting PEL\u2010PS separated patients with no negative prognostic factors (score 0: hemoglobin \u2265\u20098\u2009g\/dL and ECOG \u2264\u20092, 48.1% of patients) with median OS of 10.6\u2009years versus patients with 1\u20132 negative prognostic factors (score 1\u20132: hemoglobin &lt;\u20098\u2009g\/dL and\/or ECOG \u2265\u20093, 51.9% of patients) with median OS of 0.8\u2009years (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.0001). The PEL\u2010PS was then validated in 58 patients with HIV\u2010associated PEL treated with first\u2010line anthracycline\u2010containing chemotherapy at H\u00f4pital Saint\u2010Louis in France over the same period: median OS in patients with PEL\u2010PS 0 was 16.9\u2009years versus 0.6\u2009years in patients with PEL\u2010PS score of 1\u20132 (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.0001). The PEL\u2010PS identifies patients with good and poor prognosis. Patients with poor prognosis may benefit from novel therapies.<\/jats:p>","DOI":"10.1002\/ajh.27580","type":"journal-article","created":{"date-parts":[[2025,1,15]],"date-time":"2025-01-15T07:10:43Z","timestamp":1736925043000},"page":"393-401","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Primary Effusion Lymphoma Prognostic Score (<scp>PEL<\/scp>\u2010<scp>PS<\/scp>): A Validated International Prognostic Score in <scp>HIV<\/scp>\u2010Associated Primary Effusion Lymphoma"],"prefix":"10.1002","volume":"100","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5794-7292","authenticated-orcid":false,"given":"Kathryn","family":"Lurain","sequence":"first","affiliation":[{"name":"HIV &amp; AIDS Malignancy Branch, Center for Cancer Research, National Cancer Institute National Institutes of Health  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ramya","family":"Ramaswami","sequence":"additional","affiliation":[{"name":"HIV &amp; AIDS Malignancy Branch, Center for Cancer Research, National Cancer Institute National Institutes of Health  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8588-7138","authenticated-orcid":false,"given":"Eric","family":"Oksenhendler","sequence":"additional","affiliation":[{"name":"Department of Clinical Immunology H\u00f4pital Saint\u2010Louis, AP\u2010HP  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5006-8279","authenticated-orcid":false,"given":"David","family":"Boutboul","sequence":"additional","affiliation":[{"name":"Department of Clinical Immunology H\u00f4pital Saint\u2010Louis, AP\u2010HP  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessia","family":"Dalla Pria","sequence":"additional","affiliation":[{"name":"National Center for HIV Malignancy, Department of Oncology Chelsea &amp; Westminster Hospital  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lara","family":"Ulrich","sequence":"additional","affiliation":[{"name":"National Center for HIV Malignancy, Department of Oncology Chelsea &amp; Westminster Hospital  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Krithika","family":"Shanmugasundaram","sequence":"additional","affiliation":[{"name":"HIV &amp; AIDS Malignancy Branch, Center for Cancer Research, National Cancer Institute National Institutes of Health  Bethesda Maryland USA"},{"name":"University of Virginia  Charlottesville VA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas S.","family":"Uldrick","sequence":"additional","affiliation":[{"name":"HIV &amp; AIDS Malignancy Branch, Center for Cancer Research, National Cancer Institute National Institutes of Health  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"Bower","sequence":"additional","affiliation":[{"name":"National Center for HIV Malignancy, Department of Oncology Chelsea &amp; Westminster Hospital  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Yarchoan","sequence":"additional","affiliation":[{"name":"HIV &amp; AIDS Malignancy Branch, Center for Cancer Research, National Cancer Institute National Institutes of Health  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurence","family":"G\u00e9rard","sequence":"additional","affiliation":[{"name":"Department of Clinical Immunology H\u00f4pital Saint\u2010Louis, AP\u2010HP  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seth M.","family":"Steinberg","sequence":"additional","affiliation":[{"name":"Biostatistics and Data Management Section, Office of 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