{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,12]],"date-time":"2026-02-12T15:19:37Z","timestamp":1770909577682,"version":"3.50.1"},"reference-count":50,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2022,12,26]],"date-time":"2022-12-26T00:00:00Z","timestamp":1672012800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["acsjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Cancer"],"published-print":{"date-parts":[[2023,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Pediatric Epstein\u2010Barr virus\u2013negative monomorphic post solid organ transplant lymphoproliferative disorder [EBV(\u2212)M\u2010PTLD] comprises approximately 10% of M\u2010PTLD. No large multi\u2010institutional pediatric\u2010specific reports on treatment and outcome are available.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A multi\u2010institutional retrospective review of solid organ recipients diagnosed with EBV(\u2212)M\u2010PTLD aged \u226421\u00a0years between 2001 and 2020 in 12 centers in the United States and United Kingdom was performed, including demographics, staging, treatment, and outcomes data.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Thirty\u2010six patients were identified with EBV(\u2212)M\u2010PTLD. Twenty\u2010three (63.9%) were male. Median age (range) at transplantation, diagnosis of EBV(\u2212)M\u2010PTLD, and interval from transplant to PTLD were 2.2 years (0.1\u201317), 14 years (3.0\u201320), and 8.5 years (0.6\u201318.3), respectively. Kidney (<jats:italic>n<\/jats:italic>\u00a0=\u00a017 [47.2%]) and heart (<jats:italic>n<\/jats:italic>\u00a0=\u00a013 [36.1%]) were the most commonly transplanted organs. Most were Murphy stage III (<jats:italic>n<\/jats:italic>\u00a0=\u00a025 [69.4%]). Lactate dehydrogenase was elevated in 22\/34 (64.7%) and \u22652 times upper limit of normal in 11\/34 (32.4%). Pathological diagnoses included diffuse large B\u2010cell lymphoma (<jats:italic>n<\/jats:italic>\u00a0=\u00a031 [86.1%]) and B\u2013non\u2010Hodgkin lymphoma (B\u2010NHL) not otherwise specified (NOS) (<jats:italic>n<\/jats:italic>\u00a0=\u00a05 [13.9%]). Of nine different regimens used, the most common were: pediatric mature B\u2013NHL\u2010specific regimen (<jats:italic>n<\/jats:italic>\u00a0=\u00a013 [36.1%]) and low\u2010dose cyclophosphamide, prednisone, and rituximab (<jats:italic>n<\/jats:italic>\u00a0=\u00a09 [25%]). Median follow\u2010up from diagnosis was 3.0 years (0.3\u201311.0 years). Three\u2010year event\u2010free survival (EFS) and overall survival (OS) were 64.8% and 79.9%, respectively. Of the seven deaths, six were from progressive disease.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>EFS and OS were comparable to pediatric EBV(+) PTLD, but inferior to mature B\u2010NHL in immunocompetent pediatric patients. The wide range of therapeutic regimens used directs our work toward developing an active multi\u2010institutional registry to design prospective studies.<\/jats:p><\/jats:sec><jats:sec><jats:title>Plain Language Summary<\/jats:title><jats:p>\n<jats:list list-type=\"bullet\">\n<jats:list-item><jats:p>Pediatric Epstein\u2010Barr virus\u2013negative monomorphic post solid organ transplant lymphoproliferative disorders (EBV(\u2212)M\u2010PTLD) have comparable outcomes to EBV(+) PTLD, but are inferior to diffuse large B\u2010cell lymphoma in immunocompetent pediatric patients.<\/jats:p><\/jats:list-item>\n<jats:list-item><jats:p>The variety of treatment regimens used highlights the need to develop a pediatric PTLD registry to prospectively evaluate outcomes.<\/jats:p><\/jats:list-item>\n<jats:list-item><jats:p>The impact of treatment regimen on relapse risk could not be assessed because of small numbers.<\/jats:p><\/jats:list-item>\n<jats:list-item><jats:p>In the intensive pediatric B\u2013non\u2010Hodgkin lymphoma chemoimmunotherapy group, 11 of 13 patients remain alive in complete remission after 0.6 to 11\u00a0years.<\/jats:p><\/jats:list-item>\n<\/jats:list>\n<\/jats:p><\/jats:sec>","DOI":"10.1002\/cncr.34600","type":"journal-article","created":{"date-parts":[[2022,12,26]],"date-time":"2022-12-26T09:48:22Z","timestamp":1672048102000},"page":"780-789","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Multicenter study of pediatric Epstein\u2010Barr virus\u2013negative monomorphic post solid organ transplant lymphoproliferative disorders"],"prefix":"10.1002","volume":"129","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7238-0389","authenticated-orcid":false,"given":"Zeinab A. M.","family":"Afify","sequence":"first","affiliation":[{"name":"Primary Children's Hospital University of Utah  Salt Lake City Utah USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mary M.","family":"Taj","sequence":"additional","affiliation":[{"name":"The Royal Marsden Hospital  Sutton London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7385-7561","authenticated-orcid":false,"given":"Manuela","family":"Orjuela\u2010Grimm","sequence":"additional","affiliation":[{"name":"Columbia University Medical Center  New York New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kavitha","family":"Srivatsa","sequence":"additional","affiliation":[{"name":"The Royal Marsden Hospital  Sutton London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4250-5376","authenticated-orcid":false,"given":"Tamara P.","family":"Miller","sequence":"additional","affiliation":[{"name":"Emory University School of Medicine and Children's Healthcare of Atlanta  Atlanta Georgia USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7315-5917","authenticated-orcid":false,"given":"Holly J.","family":"Edington","sequence":"additional","affiliation":[{"name":"Emory University School of Medicine and Children's Healthcare of Atlanta  Atlanta Georgia USA"},{"name":"Novant Health Presbyterian Medical Center  Charlotte North Carolina USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mansi","family":"Dalal","sequence":"additional","affiliation":[{"name":"University of Florida Health Science Center  Gainesville Florida USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joanna","family":"Robles","sequence":"additional","affiliation":[{"name":"Duke University School of Medicine  Durham North Carolina USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"James B.","family":"Ford","sequence":"additional","affiliation":[{"name":"University of Nebraska Medical Center  Omaha Nebraska USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matthew J.","family":"Ehrhardt","sequence":"additional","affiliation":[{"name":"St. Jude Children's Research Hospital  Memphis Tennessee USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tonya J.","family":"Ureda","sequence":"additional","affiliation":[{"name":"St. Jude Children's Research Hospital  Memphis Tennessee USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeremy D.","family":"Rubinstein","sequence":"additional","affiliation":[{"name":"Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"},{"name":"University of Cincinnati College of Medicine  Cincinnati Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sarah","family":"McCormack","sequence":"additional","affiliation":[{"name":"Cincinnati Children's Hospital Medical Center  Cincinnati Ohio USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Julie M.","family":"Rivers","sequence":"additional","affiliation":[{"name":"Seattle Children's Hospital  Seattle Washington USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Karen M.","family":"Chisholm","sequence":"additional","affiliation":[{"name":"Seattle Children's Hospital  Seattle Washington USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Madison K.","family":"Kavanaugh","sequence":"additional","affiliation":[{"name":"Department of Pediatrics UPMC Children's Hospital of Pittsburgh  Pittsburgh Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrew J.","family":"Bukowinski","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Pittsburgh School of Medicine  Pittsburgh Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erika D.","family":"Friehling","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Pittsburgh School of Medicine  Pittsburgh Pennsylvania USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maegan C.","family":"Ford","sequence":"additional","affiliation":[{"name":"Columbia University Medical Center  New York New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sonika N.","family":"Reddy","sequence":"additional","affiliation":[{"name":"Columbia University Medical Center  New York New York USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lianna J.","family":"Marks","sequence":"additional","affiliation":[{"name":"Stanford University School of Medicine  Palo Alto California USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christine Moore","family":"Smith","sequence":"additional","affiliation":[{"name":"Vanderbilt University Medical Center  Nashville Tennessee USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Clinton C.","family":"Mason","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Utah  Salt Lake City Utah USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2022,12,26]]},"reference":[{"key":"e_1_2_8_2_1","first-page":"330","volume-title":"WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues","author":"Leoncini L","year":"2017"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/tp.0b013e318277e344"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1155\/2013\/814973"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1056\/nejmra1702693"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/ajt.12196"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/ajt.13324"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2016.130"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood\u20102012\u201006\u2010438549"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-3-030-11769-6_15"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1155\/2013\/150835"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/ajt.13558"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/ajt.16547"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/ctr.13652"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.v112.11.2823.2823"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.2016.69.3564"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.2967\/jnumed.121.262414"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1177\/10935266211007254"},{"issue":"3","key":"e_1_2_8_19_1","first-page":"332","article-title":"Classification, staging and end results of treatment of childhood non\u2010Hodgkin's lymphomas: dissimilarities from lymphomas in adults","volume":"7","author":"Murphy SB","year":"1980","journal-title":"Semin Oncol"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.2014.59.7203"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600\u20106143.2012.04206.x"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood\u20102006\u201007\u2010036673"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1056\/nejmoa1915315"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.16304"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/s0140\u20106736(06)67933\u20106"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1002\/cnr2.1375"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V124.21.4468.4468"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1097\/tp.0000000000002146"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1093\/ndt\/gfx356"},{"key":"e_1_2_8_30_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.2006.10.2392"},{"key":"e_1_2_8_31_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjh.17398"},{"key":"e_1_2_8_32_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277\u2010007\u20100298\u20102"},{"key":"e_1_2_8_33_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365\u20102141.2008.07149.x"},{"key":"e_1_2_8_34_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600\u20106143.2011.03896.x"},{"key":"e_1_2_8_35_1","doi-asserted-by":"publisher","DOI":"10.1038\/leu.2013.110"},{"key":"e_1_2_8_36_1","doi-asserted-by":"publisher","DOI":"10.3390\/cancers13040899"},{"key":"e_1_2_8_37_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood\u20102011\u201005\u2010349662"},{"key":"e_1_2_8_38_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.2008.20.0857"},{"key":"e_1_2_8_39_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365\u20102141.2010.08160.x"},{"key":"e_1_2_8_40_1","doi-asserted-by":"publisher","DOI":"10.1097\/tp.0b013e318269e64f"},{"key":"e_1_2_8_41_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.2009.25.4961"},{"key":"e_1_2_8_42_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.2012.43.2344"},{"key":"e_1_2_8_43_1","doi-asserted-by":"publisher","DOI":"10.1016\/s1470\u20102045(11)70300\u2010x"},{"key":"e_1_2_8_44_1","doi-asserted-by":"publisher","DOI":"10.1097\/tp.0b013e3181761659"},{"key":"e_1_2_8_45_1","unstructured":"Rituximab and LMP\u2010Specific T\u2010Cells in Treating Pediatric Solid Organ Recipients With EBV\u2010Positive CD20\u2010Positive Post\u2010Transplant Lymphoproliferative Disorder. Last updated April 30 2021. Accessed May 4 2022.https:\/\/clinicaltrials.gov\/ct2\/show\/NCT02900976"},{"key":"e_1_2_8_46_1","doi-asserted-by":"publisher","DOI":"10.1186\/s40164\u2010017\u20100087\u20100"},{"key":"e_1_2_8_47_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365\u20102141.2010.08448.x"},{"issue":"10","key":"e_1_2_8_48_1","first-page":"3945S","article-title":"A pilot study of chemoimmunotherapy (cyclophosphamide, prednisone, and rituximab) in patients with post\u2010transplant lymphoproliferative disorder following solid organ transplantation","volume":"9","author":"Orjuela M","year":"2003","journal-title":"Clin Cancer Res"},{"key":"e_1_2_8_49_1","doi-asserted-by":"publisher","DOI":"10.1056\/nejmoa011795"},{"key":"e_1_2_8_50_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1308392"},{"key":"e_1_2_8_51_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.20.00303"}],"container-title":["Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/cncr.34600","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/cncr.34600","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/acsjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/cncr.34600","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T12:38:48Z","timestamp":1760272728000},"score":1,"resource":{"primary":{"URL":"https:\/\/acsjournals.onlinelibrary.wiley.com\/doi\/10.1002\/cncr.34600"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,12,26]]},"references-count":50,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2023,3]]}},"alternative-id":["10.1002\/cncr.34600"],"URL":"https:\/\/doi.org\/10.1002\/cncr.34600","archive":["Portico"],"relation":{},"ISSN":["0008-543X","1097-0142"],"issn-type":[{"value":"0008-543X","type":"print"},{"value":"1097-0142","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,12,26]]},"assertion":[{"value":"2022-09-20","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-11-07","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-12-26","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}