{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T18:28:25Z","timestamp":1790188105997,"version":"4.1.0"},"reference-count":86,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2022,1,30]],"date-time":"2022-01-30T00:00:00Z","timestamp":1643500800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Primary effusion lymphoma (PEL) is a rare, aggressive B-cell lymphoma that usually localizes to serous body cavities to subsequently form effusions in the absence of a discrete mass. Although some tumors can develop in extracavitary locations, the areas most often affected include the peritoneum, pleural space, and the pericardium. PEL is associated with the presence of human herpesvirus 8 (HHV8), also called the Kaposi sarcoma-associated herpesvirus (KSHV), with some variability in transformation potential suggested by frequent coinfection with the Epstein-Barr virus (EBV) (~80%), although the nature of the oncogenesis is unclear. Most patients suffering with this disease are to some degree immunocompromised (e.g., Human immunodeficiency virus (HIV) infection or post-solid organ transplantation) and, even with aggressive treatment, prognosis remains poor. There is no definitive guideline for the treatment of PEL, although CHOP-like regimens (cyclophosphamide, doxorubicin, vincristine, and prednisone) are frequently prescribed and, given the rarity of this disease, therapeutic focus is being redirected to personalized and targeted approaches in the experimental realm. Current clinical trials include the combination of lenalidomide and rituximab into the EPOCH regimen and the treatment of individuals with relapsed\/refractory EBV-associated disease with tabelecleucel.<\/jats:p>","DOI":"10.3390\/cancers14030722","type":"journal-article","created":{"date-parts":[[2022,1,31]],"date-time":"2022-01-31T01:46:08Z","timestamp":1643593568000},"page":"722","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":45,"title":["Primary Effusion Lymphoma: A Clinicopathologic Perspective"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7792-3386","authenticated-orcid":false,"given":"Diamone","family":"Gathers","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, University of New Mexico, Albuquerque, NM 87131, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily","family":"Galloway","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of Arizona College of Medicine, Phoenix, AZ 85721, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katalin","family":"Kelemen","sequence":"additional","affiliation":[{"name":"Division of Hematopathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Phoenix, AZ 85054, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Allison","family":"Rosenthal","sequence":"additional","affiliation":[{"name":"Division of Hematology and Oncology, Mayo Clinic, Phoenix, AZ 85054, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Gibson","sequence":"additional","affiliation":[{"name":"Division of Hematopathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Phoenix, AZ 85054, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Javier","family":"Munoz","sequence":"additional","affiliation":[{"name":"Division of Hematology and Oncology, Mayo Clinic, Phoenix, AZ 85054, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,1,30]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"569","DOI":"10.1634\/theoncologist.12-5-569","article-title":"Primary Effusion Lymphoma","volume":"12","author":"Chen","year":"2007","journal-title":"Oncologist"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/s12885-021-08215-7","article-title":"Primary effusion lymphoma occurring in the setting of transplanted patients: A systematic review of a rare, life-threatening post-transplantation occurrence","volume":"21","author":"Zanelli","year":"2021","journal-title":"BMC Cancer"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1753","DOI":"10.1182\/blood-2019-01-893339","article-title":"Viral, immunologic, and clinical features of primary effusion lymphoma","volume":"133","author":"Lurain","year":"2019","journal-title":"Blood"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1002\/ajh.24251","article-title":"Classic and extracavitary primary effusion lymphoma in 51 HIV-infected patients from a single institution","volume":"91","author":"Guillet","year":"2016","journal-title":"Am. 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