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Though PBL is associated with human immunodeficiency virus infection and other immunosuppressed states, it can also affect immunocompetent individuals.<\/jats:p><\/jats:sec><jats:sec><jats:title>Diagnosis<\/jats:title><jats:p>The diagnosis requires a high clinical suspicion and pathological confirmation. EBER expression and <jats:italic>MYC<\/jats:italic> gene rearrangements are frequently detected. The differential diagnosis includes EBV+ diffuse large B\u2010cell lymphoma, extracavitary primary effusion lymphoma, ALK+ DLBCL, and HHV8+ large B\u2010cell lymphoma, among others.<\/jats:p><\/jats:sec><jats:sec><jats:title>Risk Stratification<\/jats:title><jats:p>Age \u226560\u2009years, advanced clinical stage, and high intermediate and high International Prognostic Index scores are associated with worse survival.<\/jats:p><\/jats:sec><jats:sec><jats:title>Management<\/jats:title><jats:p>Combination chemotherapy regimens, such as EPOCH, are recommended. The addition of bortezomib, lenalidomide, or daratumumab might improve outcomes. Including PBL patients and their participation in prospective clinical trials is warranted.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ajh.27376","type":"journal-article","created":{"date-parts":[[2024,5,20]],"date-time":"2024-05-20T09:04:20Z","timestamp":1716195860000},"page":"1586-1594","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":40,"title":["Plasmablastic lymphoma: 2024 update on diagnosis, risk stratification, and management"],"prefix":"10.1002","volume":"99","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-9637-4221","authenticated-orcid":false,"given":"Andres","family":"Ramirez\u2010Gamero","sequence":"first","affiliation":[{"name":"Division of Hematologic Malignancies Dana\u2010Farber Cancer Institute  Boston Massachusetts 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