{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T07:43:19Z","timestamp":1760168599572,"version":"build-2065373602"},"reference-count":102,"publisher":"MDPI AG","issue":"18","license":[{"start":{"date-parts":[[2021,9,8]],"date-time":"2021-09-08T00:00:00Z","timestamp":1631059200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"AIRC 5x1000","award":["21198"],"award-info":[{"award-number":["21198"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Epstein\u2013Barr virus (EBV) is a common pathogen infecting people primarily early in life. The virus has the ability to persist throughout a person\u2019s life, usually in B lymphocytes. Conditions of immunodeficiency as well as the introduction of immunosuppressive therapies and the advent of transplant technologies has brought immunodeficiency-associated lymphoproliferative disorders into view, which are often driven by EBV. The group of EBV-associated lymphoproliferative disorders includes different entities, with distinct biological features, ranging from indolent disorders, which may even spontaneously regress, to aggressive lymphomas requiring prompt and adequate treatment. These disorders are often diagnostically challenging due to their overlapping morphology and immunophenotype. Both nodal and extra-nodal sites, including the gastrointestinal tract, may be involved. This review, divided in three parts, summarizes the clinical, pathological, molecular features and treatment strategies of EBV-related lymphoproliferative disorders occurring in the gastrointestinal tract and critically analyzes the major issues in the differential diagnosis. In this part of the review, we discuss plasmablastic lymphoma, extra-cavitary primary effusion lymphoma and Burkitt lymphoma.<\/jats:p>","DOI":"10.3390\/cancers13184527","type":"journal-article","created":{"date-parts":[[2021,9,8]],"date-time":"2021-09-08T21:28:45Z","timestamp":1631136525000},"page":"4527","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["EBV-Driven Lymphoproliferative Disorders and Lymphomas of the Gastrointestinal Tract: A Spectrum of Entities with a Common Denominator (Part 2)"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8733-9933","authenticated-orcid":false,"given":"Magda","family":"Zanelli","sequence":"first","affiliation":[{"name":"Pathology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesca","family":"Sanguedolce","sequence":"additional","affiliation":[{"name":"Pathology Unit, Policlinico Riuniti, University of Foggia, 71122 Foggia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2299-5160","authenticated-orcid":false,"given":"Andrea","family":"Palicelli","sequence":"additional","affiliation":[{"name":"Pathology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9841-7856","authenticated-orcid":false,"given":"Maurizio","family":"Zizzo","sequence":"additional","affiliation":[{"name":"Surgical Oncology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giovanni","family":"Martino","sequence":"additional","affiliation":[{"name":"Pathology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, 05100 Terni, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cecilia","family":"Caprera","sequence":"additional","affiliation":[{"name":"Pathology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, 05100 Terni, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4189-0316","authenticated-orcid":false,"given":"Valentina","family":"Fragliasso","sequence":"additional","affiliation":[{"name":"Laboratory of Translational Research, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alessandra","family":"Soriano","sequence":"additional","affiliation":[{"name":"Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA"},{"name":"Gastroenterology Division, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luca","family":"Valle","sequence":"additional","affiliation":[{"name":"Anatomic Pathology, Department of Integrated Surgical and Diagnostic Sciences (DISC), University of Genoa and Ospedale Policlinico San Martino, 16132 Genoa, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefano","family":"Ricci","sequence":"additional","affiliation":[{"name":"Pathology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4147-5534","authenticated-orcid":false,"given":"Fabrizio","family":"Gozzi","sequence":"additional","affiliation":[{"name":"Ocular Immunology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luca","family":"Cimino","sequence":"additional","affiliation":[{"name":"Ocular Immunology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alberto","family":"Cavazza","sequence":"additional","affiliation":[{"name":"Pathology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesco","family":"Merli","sequence":"additional","affiliation":[{"name":"Hematology Unit, Azienda USL-IRCCS di Reggio Emilia, 42123 Reggio Emilia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefano A.","family":"Pileri","sequence":"additional","affiliation":[{"name":"Haematopathology Division, European Institute of Oncology-IEO IRCCS Milan, 20141 Milan, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefano","family":"Ascani","sequence":"additional","affiliation":[{"name":"Pathology Unit, Azienda Ospedaliera Santa Maria di Terni, University of Perugia, 05100 Terni, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2021,9,8]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1146\/annurev-immunol-032414-112326","article-title":"The immunology of Epstein-Barr virus induced disease","volume":"33","author":"Taylor","year":"2015","journal-title":"Annu. Rev. Immunol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"789","DOI":"10.1038\/nrc.2016.92","article-title":"Epstein-Barr virus: More than 50 years old and still providing surprises","volume":"16","author":"Young","year":"2016","journal-title":"Nat. Rev. Cancer"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1293","DOI":"10.1016\/j.humpath.2007.05.020","article-title":"Epstein-Barr virus-associated lymphoproliferative disorders","volume":"38","author":"Resk","year":"2007","journal-title":"Hum. Pathol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1034\/j.1399-3062.2001.003002060.x","article-title":"The Epstein-Barr virus and post-transplant lymphoproliferative disease: Interplay of immunosuppression, EBV, and the immune system in disease pathogenesis","volume":"3","author":"Tanner","year":"2001","journal-title":"Transpl. Infect. Dis."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1111\/j.1399-0039.2007.00891.x","article-title":"Immunosenescence: Deficits in adaptative immunity in the elderly","volume":"70","author":"Hakim","year":"2007","journal-title":"Tissue Antigens"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"780","DOI":"10.1111\/j.1365-2141.2007.06867.x","article-title":"Age-dependent accumulation of monoclonal CD4+ CD8+ double positive T lymphocytes in the peripheral blood of the elderly","volume":"139","author":"Ghia","year":"2007","journal-title":"Br. J. Haematol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"441","DOI":"10.1016\/j.semcancer.2013.07.005","article-title":"Interplay among viral antigens, cellular pathways and tumor microenvironment in the pathogenesis of EBV-driven lymphoma","volume":"13","author":"Dolcetti","year":"2013","journal-title":"Semin. Cancer Biol."},{"key":"ref_8","unstructured":"Swerdlow, S.H., Campo, E., Harris, N.L., Jaffe, E.S., Pileri, S.A., Stein, H., and Thiele, J. (2017). WHO Classification of Tumours Haematopoietic and Lymphoid Tissues, IARC. [4th ed.]."},{"key":"ref_9","doi-asserted-by":"crossref","unstructured":"Dojcinov, S.D., Fend, F., and Quintanilla-Martinez, L. (2021). EBV-positive lymphoproliferation of B-T- and NK-cell derivation in non-immunocompromised hosts. Pathogens, 7.","DOI":"10.3390\/pathogens7010028"},{"key":"ref_10","first-page":"151","article-title":"Infectious mononucleosis","volume":"390","author":"Dunmire","year":"2015","journal-title":"Curr. Top. Microbiol."},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"Rickinson, A.B. (2014). Co-infections, inflammation and oncogenesis: Future directions for EBV research. Seminars in Cancer Biology, Academic Press.","DOI":"10.1016\/j.semcancer.2014.04.004"},{"key":"ref_12","doi-asserted-by":"crossref","unstructured":"Price, A.M., and Luftig, M.A. (2015). To be or not IIb: A multi-step process for Epstein-Barr virus latency establishment and consequences for B cell tumorigenesis. PLoS Pathog., 11.","DOI":"10.1371\/journal.ppat.1004656"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1097\/PAS.0b013e3181cf8622","article-title":"EBV positive mucocutaneous ulcer. A study of 26 cases associated with various sources of immunosuppression","volume":"34","author":"Dojcinov","year":"2010","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"64","DOI":"10.3960\/jslrt.18039","article-title":"A review of EBV-positive mucocutaneous ulcers focusing on clinical and pathological aspects","volume":"59","author":"Ikeda","year":"2019","journal-title":"J. Clin. Exp. Hematopath."},{"key":"ref_15","doi-asserted-by":"crossref","unstructured":"Ikeda, T., Gion, Y., Nishimura, M.F., Yoshino, T., and Sato, Y. (2021). Epstein-Barr Virus-positive mucocutaneous ulcer: A unique and curious disease entity. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms22031053"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1093\/ajcp\/aqw214","article-title":"EBV-positive B-cell proliferations of varied malignant potential","volume":"147","author":"Natkunam","year":"2017","journal-title":"Am. J. Clin. Pathol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1522","DOI":"10.1097\/PAS.0000000000000282","article-title":"EBV-positive mucocutaneous ulcer in organ transplant recipients: A localized indolent posttransplant lymphoproliferative disorder","volume":"38","author":"Hart","year":"2014","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"1747","DOI":"10.1016\/j.crohns.2014.08.010","article-title":"Azathioprine induced Epstein Barr virus-positive mucocutaneous ulcer arising in perianal fistula and abscess associated with Crohn\u2019s disease","volume":"8","author":"Matnani","year":"2014","journal-title":"J. Crohn\u2019s Colitis"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"6072","DOI":"10.3748\/wjg.v21.i19.6072","article-title":"Epstein Barr virus-positive mucocutaneous ulcer of the colon associated Hodgkin lymphoma in Crohn\u2019s disease","volume":"21","author":"Moran","year":"2015","journal-title":"World J. Gastroenterol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"934","DOI":"10.1016\/j.dld.2017.03.011","article-title":"Epstein-Barr virus-positive mucocutaneous ulcer in Crohn\u2019s disease. A condition to consider in immunosuppressed IBD patients","volume":"49","author":"Juan","year":"2017","journal-title":"Dig. Liver Dis."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1007\/s00428-018-2451-9","article-title":"Primary classic Hodgkin lymphoma of the ileum and Epstein-Barr virus mucocutaneous ulcer of the colon: Two entities compared","volume":"474","author":"Zanelli","year":"2019","journal-title":"Virchows Arch."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"810","DOI":"10.1016\/j.jaip.2014.07.009","article-title":"A rare presentation of EBV+ mucocutaneous ulcer that led to the diagnosis of hypogammaglobulinemia","volume":"2","author":"Kleinman","year":"2014","journal-title":"J. Allergy Clin. Immunol. Pract."},{"key":"ref_23","doi-asserted-by":"crossref","unstructured":"Osman, M., Al Salihi, M., Abu Sitta, E., and Al Hadidi, S. (2017). A rare case of Epstein-Barr virus mucocutaneous ulcer of the colon. BMJ Case Rep.","DOI":"10.1136\/bcr-2017-220717"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1795","DOI":"10.1007\/s00277-019-03712-z","article-title":"EBV-positive mucocutaneous ulcer within colonic diverticulitis mimicking diffuse large B-cell lymphoma","volume":"98","author":"Zanelli","year":"2019","journal-title":"Ann. Hematol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1053\/j.semdp.2021.04.004","article-title":"Rare EBV-associated B cell neoplasms of the gastrointestinal tract","volume":"38","author":"Volaric","year":"2021","journal-title":"Semin. Diagn. Pathol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"2437","DOI":"10.1038\/s41379-020-0599-8","article-title":"Clinicopathological analysis of 34 Japanese patients with EBV-positive mucocutaneous ulcer","volume":"33","author":"Ikeda","year":"2020","journal-title":"Mod. Pathol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1097\/00000478-200001000-00008","article-title":"Epstein-Barr virus-positive primary gastrointestinal Hodgkin\u2019s disease: Association with inflammatory bowel disease and immunosuppression","volume":"24","author":"Kumar","year":"2000","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.leukres.2018.06.008","article-title":"Hodgkin lymphoma of the gastrointestinal tract in patients with inflammatory bowel disease: Portrait of a rare entity","volume":"71","author":"Barzilai","year":"2018","journal-title":"Leuk. Res."},{"key":"ref_29","first-page":"219","article-title":"Syncitial variant of nodular sclerosis classical Hodgkin lymphoma of the terminal ileum in a patient with longstanding Crohn\u2019s disease","volume":"46","author":"Gibson","year":"2016","journal-title":"Ann. Clin. Lab. Sci."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"745","DOI":"10.1016\/j.path.2019.03.002","article-title":"EBV-associated lymphoproliferative disorders: Update in classification","volume":"12","author":"Rezk","year":"2019","journal-title":"Surg. Pathol."},{"key":"ref_31","first-page":"411","article-title":"Endemic Burkitt\u2019s lymphoma as a polymicrobial disease: New insights on the interaction between Plasmodium falciparum and Epstein-Barr virus","volume":"19","author":"Chen","year":"2009","journal-title":"Semin. Cancer Biol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"6963","DOI":"10.1158\/0008-5472.CAN-08-0627","article-title":"EBV is necessary for proliferation of dually infected primary effusion lymphoma cells","volume":"68","author":"Mack","year":"2008","journal-title":"Cancer Res."},{"key":"ref_33","unstructured":"Swerdlow, S.H., Campo, E., Harris, N.L., Jaffe, E.S., Pileri, S.A., Stein, H., and Thiele, J. (2008). WHO Classification of Tumours of Haematopoietic and Lymphoid Tissue, IARC."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"1413","DOI":"10.1182\/blood.V89.4.1413","article-title":"Plasmablastic lymphomas of the oral cavity: A new entity associated with the human immunodeficiency virus infection","volume":"89","author":"Delecleuse","year":"1997","journal-title":"Blood"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"e22335","DOI":"10.1097\/MD.0000000000022335","article-title":"Oral plasmablastic lymphoma: A case report","volume":"99","author":"Zizzo","year":"2020","journal-title":"Medicine"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"875","DOI":"10.1097\/PAS.0000000000000234","article-title":"Clinicopathologic comparison of plasmablastic lymphoma in HIV-positive, immunocompetent and posttransplant patients single-center series of 25 cases and meta-analysis of 277 reported cases","volume":"38","author":"Morscio","year":"2014","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"2323","DOI":"10.1182\/blood-2014-10-567479","article-title":"The biology and treatment of plasmablastic lymphoma","volume":"125","author":"Castillo","year":"2015","journal-title":"Blood"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"1074","DOI":"10.5858\/arpa.2016-0232-RA","article-title":"Plasmablastic lymphoma: A review of clinicopathologic features and differential diagnosis","volume":"140","author":"Harmon","year":"2016","journal-title":"Arch. Pathol. Lab. Med."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"152973","DOI":"10.1016\/j.prp.2020.152973","article-title":"Clinical, pathological and molecular features of plasmablastic lymphoma arising in the gastrointestinal tract: A review and reappraisal","volume":"216","author":"Sanguedolce","year":"2020","journal-title":"Pathol. Res. Pract."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"1177","DOI":"10.1101\/gr.157743.113","article-title":"Large-scale hypomethylated blocks associated with Epstein-Barr virus-induced B-cell immortalization","volume":"24","author":"Hansen","year":"2014","journal-title":"Genome Res."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"63","DOI":"10.2147\/BLCTT.S142814","article-title":"Plasmablastic lymphoma: Current perspectives","volume":"8","author":"Lopez","year":"2018","journal-title":"Blood Lymph. Cancer Targets Ther."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1111\/his.12596","article-title":"Recent advances in intestinal lymphomas","volume":"66","author":"Foukas","year":"2015","journal-title":"Histopathology"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"11894","DOI":"10.3748\/wjg.v20.i33.11894","article-title":"Manifestations of gastrointestinal plasmablastic lymphoma: A case series with literature review","volume":"20","author":"Luria","year":"2014","journal-title":"World J. Gastroenterol."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1586\/eci.10.36","article-title":"Update on the risk of lymphoma following immunosuppressive therapy for inflammatory bowel disease","volume":"6","author":"Bewtra","year":"2010","journal-title":"Expert Rev. Clin. Immunol."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1111\/jgh.12015","article-title":"Lymphoma and other lymphoproliferative disorders in inflammatory bowel disease: A review","volume":"28","author":"Subramaniam","year":"2013","journal-title":"J. Gastroenterol. Hepatol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1097\/PAT.0000000000000039","article-title":"Plasmablastic lymphoma presenting as a colonic stricture in Crohn\u2019s disease","volume":"46","author":"Liu","year":"2014","journal-title":"Pathology"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1030","DOI":"10.1016\/j.prp.2015.09.012","article-title":"Unique presentation of a plasmablastic lymphoma superficially involving the entire large bowel","volume":"211","author":"Zanelli","year":"2015","journal-title":"Pathol. Res. Pract."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"622","DOI":"10.1046\/j.1365-2141.2002.03872.x","article-title":"Molecular histogenesis of plasmablastic lymphoma of the oral cavity","volume":"119","author":"Gaidano","year":"2002","journal-title":"Br. J. Haematol."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"1686","DOI":"10.1097\/PAS.0b013e3181f3e29f","article-title":"IG\/MYC rearrangements are the main cytogenetic alteration in plasmablastic lymphomas","volume":"34","author":"Valera","year":"2010","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1111\/his.14336","article-title":"MYC status in HIV-associated plasmablastic lymphoma: Dual colour CISH, FISH and immunohistochemistry","volume":"79","author":"Pather","year":"2021","journal-title":"Histopathology"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1038\/modpathol.2016.162","article-title":"Plasmablastic lymphoma phenotype is determined by genetic alterations in MYC and PRDM1","volume":"30","author":"Linares","year":"2017","journal-title":"Mod. Pathol."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"953","DOI":"10.1002\/ajh.25112","article-title":"EBV-positive large B-cell lymphoma, not otherwise specified: 2018 update on diagnosis, risk-stratification and management","volume":"93","author":"Castillo","year":"2018","journal-title":"Am. J. Hematol."},{"key":"ref_53","doi-asserted-by":"crossref","unstructured":"Ishikawa, E., Satou, A., Nakamura, M., Nakamura, S., and Fujishiro, M. (2021). Epstein-Barr virus positive B-cell lymphoproliferative disorder of the gastrointestinal tract. Cancers, 13.","DOI":"10.3390\/cancers13153815"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"1547","DOI":"10.1016\/j.leukres.2011.06.036","article-title":"Plasmablastic lymphoma: Are more intensive regimens needed?","volume":"35","author":"Castillo","year":"2011","journal-title":"Leuk. Res."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"967","DOI":"10.1007\/s00277-016-2904-7","article-title":"Refractory plasmablastic lymphoma. A review of treatment options beyond standard therapy","volume":"96","author":"Pretscher","year":"2017","journal-title":"Ann. Hematol."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"352","DOI":"10.1111\/bjh.13300","article-title":"Bortezomib in combination with infusional dose-adjusted EPOCH for the treatment of plasmablastic lymphoma","volume":"169","author":"Castillo","year":"2015","journal-title":"Br. J. Haematol."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"1877","DOI":"10.1016\/j.bbmt.2014.06.009","article-title":"Hematopoietic cell transplantation for plasmablastic lymphoma: A review","volume":"20","author":"Castillo","year":"2014","journal-title":"Biol. Blood Marrow Transplant."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1097\/PAP.0000000000000253","article-title":"Lymphoid neoplasms with plasmablastic differentiation: A comprehensive review and diagnostic approaches","volume":"27","author":"Chen","year":"2020","journal-title":"Adv. Anat. Pathol."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1186\/s13045-015-0163-z","article-title":"Stage, age and EBV status impact outcomes of plasmablastic lymphoma patients: A clinicopathologic analysis of 61 patients","volume":"8","author":"Loghavi","year":"2015","journal-title":"J. Hematol. Oncol."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"976","DOI":"10.3324\/haematol.2016.141978","article-title":"Immune-checkpoint expression in Epstein-Barr virus positive and negative plasmablastic lymphoma: A clinical and pathological study in 82 patients","volume":"101","author":"Laurent","year":"2016","journal-title":"Haematologica"},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1177\/1066896914536223","article-title":"Anaplastic lymphoma kinase-positive large B-cell lymphoma: Description of a case with an unexpected clinical outcome","volume":"23","author":"Zanelli","year":"2015","journal-title":"Int. J. Surg. Pathol."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1177\/1066896917750471","article-title":"Multiple myeloma with multilobated plasma cells: An unusual and challenging morphological variant","volume":"26","author":"Zanelli","year":"2018","journal-title":"Int. J. Surg. Pathol."},{"key":"ref_63","doi-asserted-by":"crossref","unstructured":"Zanelli, M., Ricci, S., Zizzo, M., Sanguedolce, F., De Giorgi, F., Palicelli, A., Martino, G., and Ascani, S. (2021). Systemic mastocytosis associated with \u201csmoldering\u201d multiple myeloma. Diagnostics, 11.","DOI":"10.3390\/diagnostics11010088"},{"key":"ref_64","doi-asserted-by":"crossref","unstructured":"Zanelli, M., Sanguedolce, F., Zizzo, M., Palicelli, A., Bassi, M.C., Santandrea, G., Martino, G., Soriano, A., Caprera, C., and Corsi, M. (2021). Primary effusion lymphoma occurring in the setting of transplanted patients: A systematic review of a rare, life-threatening post-transplantation occurrence. BMC Cancer, 21.","DOI":"10.1186\/s12885-021-08215-7"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"1401","DOI":"10.1097\/01.pas.0000138177.10829.5c","article-title":"KSHV-positive solid lymphomas represent an extra-cavitary variant of primary effusion lymphoma","volume":"28","author":"Chadburn","year":"2004","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"441","DOI":"10.1016\/j.anndiagpath.2012.03.004","article-title":"Extracavitary\/solid variant of primary effusion lymphoma","volume":"16","author":"Kim","year":"2012","journal-title":"Ann. Diagn. Pathol."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"1129","DOI":"10.1097\/PAS.0b013e31825b38ec","article-title":"Extracavitary KSHV-associated large B-cell lymphoma: A distinct entity or a subtype of primary effusion lymphoma? Study of 9 cases and review of an additional 43 cases","volume":"36","author":"Pan","year":"2012","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"846","DOI":"10.1053\/hupa.2002.126184","article-title":"Human herpes-virus-8-associated lymphoma of the bowel in human immunodeficiency virus-positive patients without history of primary effusion lymphoma","volume":"33","author":"Costes","year":"2002","journal-title":"Hum. Pathol."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"630","DOI":"10.1016\/S0046-8177(03)00093-5","article-title":"Anorectal lymphoma without effusion associated with human herpesvirus-8 and type 1 Epstein-Barr virus in an HIV infected patient","volume":"34","author":"Navarro","year":"2003","journal-title":"Hum. Pathol."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1016\/j.yexmp.2015.09.010","article-title":"Extracavitary\/solid variant of primary effusion lymphoma presenting as a gastric mass","volume":"99","author":"Liao","year":"2015","journal-title":"Exp. Mol. Pathol."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"149","DOI":"10.3816\/CLM.2005.n.044","article-title":"Extracavitary primary effusion lymphoma of the anorectum","volume":"6","author":"Pantanowitz","year":"2005","journal-title":"Clin. Lymphoma Myeloma"},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1111\/bjh.16196","article-title":"Extracavitary primary effusion lymphoma in a post-transplantation patient","volume":"187","author":"Zanelli","year":"2019","journal-title":"Br. J. Hematol."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"719","DOI":"10.1097\/00000478-199706000-00013","article-title":"Kaposi\u2019s sarcoma-associated herpesvirus and human herpesvirus 8 (KSHV\/HHV8)-associated lymphoma of the bowel. Report of two cases in HIV-positive men with secondary effusion lymphomas","volume":"21","author":"DePond","year":"1997","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"1363","DOI":"10.1097\/00000478-200210000-00014","article-title":"Primary effusion lymphoma with subsequent development of a small bowel mass in an HIV-seropositive patient: A case report and literature review","volume":"26","author":"Huang","year":"2002","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1016\/j.humpath.2018.02.020","article-title":"Kaposi sarcoma-associated herpesvirus\/human herpesvirus 8-associated extracavitary primary effusion lymphoma presenting as multiple lymphomatous polyposis","volume":"79","author":"Oster","year":"2018","journal-title":"Hum. Pathol."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"e13518","DOI":"10.1111\/ctr.13518","article-title":"Human herpesvirus 6, 7, and 8 in solid organ transplantation: Guidelines from the American Society of Transplantation Infectious Diseases Community of Practice","volume":"33","author":"Hand","year":"2019","journal-title":"Clin. Transplant."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"1337","DOI":"10.1016\/S0140-6736(96)90988-5","article-title":"HHV8 DNA in normal gastrointestinal mucosa from HIV seropositive people","volume":"37","author":"Thomas","year":"1996","journal-title":"Lancet"},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"1865","DOI":"10.1126\/science.7997879","article-title":"Identification of herpesvirus-like DNA sequences in AIDS-associated Kaposi\u2019s sarcoma","volume":"266","author":"Chang","year":"1994","journal-title":"Science"},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"1276","DOI":"10.1182\/blood.V86.4.1276.bloodjournal8641276","article-title":"Kaposi\u2019s sarcoma-associated herpesvirus-like DNA sequences in multicentric Castleman\u2019s disease","volume":"86","author":"Soulier","year":"1995","journal-title":"Blood"},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"1186","DOI":"10.1056\/NEJM199505043321802","article-title":"Kaposi\u2019s sarcoma-associated herpesvirus-like DNA sequences in AIDS-related body cavity-based lymphomas","volume":"332","author":"Cesarman","year":"1995","journal-title":"N. Engl. J. Med."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"3415","DOI":"10.1182\/blood-2002-02-0487","article-title":"KSHV and EBV-associated germinotropic lymphoproliferative disorder","volume":"100","author":"Du","year":"2002","journal-title":"Blood"},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"2243","DOI":"10.1007\/s00277-020-04024-3","article-title":"Germinotropic lymphoproliferative disorder: A systematic review","volume":"99","author":"Zanelli","year":"2020","journal-title":"Ann. Hematol."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"2439","DOI":"10.1007\/s00277-019-03773-0","article-title":"HHV-8- and EBV-positive germinotropic lymphoproliferative disorder","volume":"98","author":"Zanelli","year":"2019","journal-title":"Ann. Hematol."},{"key":"ref_84","doi-asserted-by":"crossref","unstructured":"Zanelli, M., Mengoli, M.C., Del Sordo, R., Cagini, A., DE Marco, L., Simonetti, E., Martino, G., Zizzo, M., and Ascani, S. (2018). Intravascular NK\/T-cell lymphoma, Epstein-Barr virus positive with multiorgan involvement; a clinical dilemma. BMC Cancer, 18.","DOI":"10.1186\/s12885-018-5001-6"},{"key":"ref_85","doi-asserted-by":"crossref","unstructured":"Zanelli, M., Stingeni, L., Zizzo, M., Martino, G., Sanguedolce, F., Marra, A., Crescenzi, B., and Pileri, S.A. (2021). HHV8-positive Castleman disease and in situ mantle cell neoplasia within dermatopathic lymphadenitis, in longstanding psoriasis. Diagnostics, 11.","DOI":"10.3390\/diagnostics11071150"},{"key":"ref_86","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_87","doi-asserted-by":"crossref","first-page":"65","DOI":"10.5582\/irdr.2014.01010","article-title":"Current status of treatment for primary effusion lymphoma","volume":"3","author":"Okada","year":"2014","journal-title":"Intractable Rare Dis. Res."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1002\/bjs.18004619704","article-title":"A sarcoma involving the jaw of African children","volume":"46","author":"Burkitt","year":"1958","journal-title":"Br. J. Surg."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"702","DOI":"10.1016\/S0140-6736(64)91524-7","article-title":"Virus particle in cultured lymphoblasts from Burkitt\u2019s lymphoma","volume":"1","author":"Epstein","year":"1964","journal-title":"Lancet"},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"756","DOI":"10.1038\/274756a0","article-title":"Epidemiological evidence for causal relationship between Epstein-Barr virus and Burkitt\u2019s lymphoma from Ugandan prospective study","volume":"274","author":"Geser","year":"1978","journal-title":"Nature"},{"key":"ref_91","first-page":"494758","article-title":"A role for RNA viruses in the pathogenesis of Burkitt\u2019s lymphoma: The need for reappraisal","volume":"2012","year":"2012","journal-title":"Adv. Hematol."},{"key":"ref_92","doi-asserted-by":"crossref","unstructured":"Abate, F., Ambrosio, M.R., Mundo, L., Laginestra, M.A., Fulingni, F., Rossi, M., Zairis, S., Gazaneo, S., De Falco, G., and Lazzi, S. (2015). Distinct viral and mutational spectrum of endemic Burkitt lymphoma. PLoS Pathog., 11.","DOI":"10.1371\/journal.ppat.1005158"},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1016\/S0140-6736(77)91137-0","article-title":"Is Burkitt\u2019s lymphoma related to perinatal infection by Epstein-Barr virus?","volume":"309","year":"1977","journal-title":"Lancet"},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1097\/PAS.0000000000000332","article-title":"Epstein-Barr virus (EBV)-positive sporadic Burkitt lymphoma: And age-related lymphoproliferative disorder?","volume":"39","author":"Satou","year":"2015","journal-title":"Am. J. Surg. Pathol."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"573","DOI":"10.1002\/ajh.23187","article-title":"Pediatric, elderly and emerging adult-onset peaks in Burkitt\u2019s lymphoma incidence diagnosed in four continents, excluding Africa","volume":"87","author":"Mbulaiteye","year":"2012","journal-title":"Am. J. Hematol."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"2313","DOI":"10.1097\/QAD.0000000000000428","article-title":"Risk of non-Hodgkin lymphoma subtypes in HIV-infected people during the HAART era: A population-based study","volume":"28","author":"Gibson","year":"2014","journal-title":"AIDS"},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"1234","DOI":"10.1016\/S0140-6736(11)61177-X","article-title":"Burkitt\u2019s lymphoma","volume":"379","author":"Molyneux","year":"2012","journal-title":"Lancet"},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1007\/s00383-011-2982-5","article-title":"Intussusception as a presenting feature of Burkitt lymphoma: Implications for management and outcome","volume":"28","author":"England","year":"2012","journal-title":"Pediatr. Surg. Int."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1016\/j.semcancer.2009.07.004","article-title":"Burkitt\u2019s lymphoma: The Rosetta stone deciphering Epstein-Barr virus biology","volume":"19","author":"Rowe","year":"2009","journal-title":"Semin. Cancer Biol."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1016\/j.semcancer.2009.07.007","article-title":"How does Epstein-Barr virus (EBV) complement the activation of MYC in the pathogenesis of Burkitt\u2019s lymphoma?","volume":"19","author":"Allday","year":"2009","journal-title":"Semin. Cancer Biol."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"1333","DOI":"10.1016\/j.hoc.2016.07.009","article-title":"Update on Burkitt lymphoma","volume":"30","author":"Duleavy","year":"2016","journal-title":"Hematol. Oncol. Clin. North Am."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1046\/j.1365-2257.2002.00429.x","article-title":"Burkitt\u2019s lymphoma: Single centre experience with modified BMF protocol","volume":"24","author":"Harris","year":"2002","journal-title":"Clin. Lab. Haematol."}],"container-title":["Cancers"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2072-6694\/13\/18\/4527\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T06:59:07Z","timestamp":1760165947000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2072-6694\/13\/18\/4527"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,9,8]]},"references-count":102,"journal-issue":{"issue":"18","published-online":{"date-parts":[[2021,9]]}},"alternative-id":["cancers13184527"],"URL":"https:\/\/doi.org\/10.3390\/cancers13184527","relation":{},"ISSN":["2072-6694"],"issn-type":[{"type":"electronic","value":"2072-6694"}],"subject":[],"published":{"date-parts":[[2021,9,8]]}}}