{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,10]],"date-time":"2026-11-10T18:03:25Z","timestamp":1794333805284,"version":"4.9.0"},"reference-count":30,"publisher":"American Society of Clinical Oncology (ASCO)","issue":"2","content-domain":{"domain":["ascopubs.org"],"crossmark-restriction":true},"short-container-title":["JCO"],"published-print":{"date-parts":[[2015,1,10]]},"abstract":"<jats:sec>\n                    <jats:title>Purpose<\/jats:title>\n                    <jats:p>Biallelic mutations in ATM cause ataxia-telangiectasia (AT), a rare inherited disease with a high incidence of cancer. Precise estimates of the risk, presentation, and outcomes of cancer in patients with AT need to be addressed in large series.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Patients and Methods<\/jats:title>\n                    <jats:p>In this large retrospective cohort, 69 patients with cancers (24.5%) were identified among 279 patients with AT. Centralized review was performed on 60% of the lymphomas. Incidence rates were compared with the French population, and risk factors were analyzed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Eight patients developed acute leukemias (including four T-cell acute lymphoblastic leukemias), 12 developed Hodgkin lymphoma (HL), 38 developed non-Hodgkin lymphoma (NHL), three developed T-cell prolymphocytic leukemia (T-PLL), and eight developed carcinoma at a median age of 8.3, 10.6, 9.7, 24.2, and 31.4 years, respectively (P &lt; .001). The majority of NHLs were aggressive B-cell NHL. Epstein-Barr virus was associated with all of the HLs and 50% of the NHLs. Overall survival was shorter in patients with AT who developed cancer compared with those who did not develop cancer (15 v 24 years, respectively; P &lt; .001). Survival was improved in patients who achieved a major response to treatment (3.46 v 0.87 years for major v minor responses, respectively; P = .011). Immunodeficiency was associated with increased risk of cancer. ATM mutation type was associated with a difference in survival in the entire cohort but not with cancer incidence or cancer survival.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>B-cell NHL, HL, and acute lymphoblastic leukemia occur at a high rate and earlier age than carcinomas in AT. T-PLLs are rarer than initially reported. Prognosis is poor, but patients may benefit from treatment with an improved survival.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1200\/jco.2014.56.5101","type":"journal-article","created":{"date-parts":[[2014,12,9]],"date-time":"2014-12-09T01:04:16Z","timestamp":1418087056000},"page":"202-208","update-policy":"https:\/\/doi.org\/10.1200\/crossmark","source":"Crossref","is-referenced-by-count":230,"title":["Incidence, Presentation, and Prognosis of Malignancies in Ataxia-Telangiectasia: A Report From the French National Registry of Primary Immune Deficiencies"],"prefix":"10.1200","volume":"33","author":[{"given":"Felipe","family":"Suarez","sequence":"first","affiliation":[{"name":"Felipe Suarez, Nizar Mahlaoui, Danielle Canioni, Nicole Brousse, Jean-Philippe Jais, Alain Fischer, and Olivier Hermine, H\u00f4pital Universitaire Necker\u2013Enfants Malades, Assistance Publique\u2013H\u00f4pitaux de Paris; Felipe Suarez, Nizar Mahlaoui, Chantal Andriamanga, Alain Fischer, and Olivier Hermine, French National Reference Center for Primary Immune Deficiency; Felipe Suarez, Nizar Mahlaoui, Jean-Philippe Jais, Alain Fischer, and Olivier Hermine, Imagine Institute, Institut National de la Recherche..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nizar","family":"Mahlaoui","sequence":"additional","affiliation":[{"name":"Felipe Suarez, Nizar Mahlaoui, Danielle Canioni, Nicole Brousse, Jean-Philippe Jais, Alain Fischer, and Olivier Hermine, H\u00f4pital Universitaire Necker\u2013Enfants Malades, Assistance Publique\u2013H\u00f4pitaux de Paris; Felipe Suarez, Nizar Mahlaoui, Chantal Andriamanga, Alain Fischer, and Olivier Hermine, French National Reference Center for Primary Immune Deficiency; Felipe Suarez, Nizar Mahlaoui, Jean-Philippe Jais, Alain Fischer, and Olivier Hermine, Imagine Institute, Institut National de la Recherche..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Danielle","family":"Canioni","sequence":"additional","affiliation":[{"name":"Felipe Suarez, Nizar Mahlaoui, Danielle Canioni, Nicole Brousse, Jean-Philippe Jais, Alain Fischer, and Olivier Hermine, H\u00f4pital Universitaire Necker\u2013Enfants Malades, Assistance Publique\u2013H\u00f4pitaux de Paris; Felipe Suarez, Nizar Mahlaoui, Chantal Andriamanga, Alain Fischer, and Olivier Hermine, French National Reference Center for Primary Immune Deficiency; Felipe Suarez, Nizar Mahlaoui, Jean-Philippe Jais, Alain Fischer, and Olivier Hermine, Imagine Institute, Institut National de la Recherche..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chantal","family":"Andriamanga","sequence":"additional","affiliation":[{"name":"Felipe Suarez, Nizar Mahlaoui, Danielle Canioni, Nicole Brousse, Jean-Philippe Jais, Alain Fischer, and Olivier Hermine, H\u00f4pital Universitaire Necker\u2013Enfants Malades, Assistance Publique\u2013H\u00f4pitaux de Paris; Felipe Suarez, Nizar Mahlaoui, Chantal Andriamanga, Alain Fischer, and Olivier Hermine, French National Reference Center for Primary Immune Deficiency; Felipe Suarez, Nizar Mahlaoui, Jean-Philippe Jais, Alain Fischer, and Olivier Hermine, Imagine Institute, Institut National de la Recherche..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"Dubois d'Enghien","sequence":"additional","affiliation":[{"name":"Felipe Suarez, Nizar Mahlaoui, Danielle Canioni, Nicole Brousse, Jean-Philippe Jais, Alain Fischer, and Olivier Hermine, H\u00f4pital Universitaire Necker\u2013Enfants Malades, Assistance Publique\u2013H\u00f4pitaux de Paris; Felipe Suarez, Nizar Mahlaoui, Chantal Andriamanga, Alain Fischer, and Olivier Hermine, French National Reference Center for Primary Immune Deficiency; Felipe Suarez, Nizar Mahlaoui, Jean-Philippe Jais, Alain Fischer, and Olivier Hermine, Imagine Institute, Institut National de la Recherche..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole","family":"Brousse","sequence":"additional","affiliation":[{"name":"Felipe Suarez, Nizar Mahlaoui, Danielle Canioni, Nicole Brousse, Jean-Philippe Jais, Alain Fischer, and Olivier Hermine, H\u00f4pital Universitaire Necker\u2013Enfants Malades, Assistance Publique\u2013H\u00f4pitaux de Paris; Felipe Suarez, Nizar Mahlaoui, Chantal Andriamanga, Alain Fischer, and Olivier Hermine, French National Reference Center for Primary Immune Deficiency; Felipe Suarez, Nizar Mahlaoui, Jean-Philippe Jais, Alain Fischer, and Olivier Hermine, Imagine Institute, Institut National de la Recherche..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Philippe","family":"Jais","sequence":"additional","affiliation":[{"name":"Felipe Suarez, Nizar Mahlaoui, Danielle Canioni, Nicole Brousse, Jean-Philippe Jais, Alain Fischer, and Olivier Hermine, H\u00f4pital Universitaire Necker\u2013Enfants Malades, Assistance Publique\u2013H\u00f4pitaux de Paris; Felipe Suarez, Nizar Mahlaoui, Chantal Andriamanga, Alain Fischer, and Olivier Hermine, French National Reference Center for Primary Immune Deficiency; Felipe Suarez, Nizar Mahlaoui, Jean-Philippe Jais, Alain Fischer, and Olivier Hermine, Imagine Institute, Institut National de la Recherche..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alain","family":"Fischer","sequence":"additional","affiliation":[{"name":"Felipe Suarez, Nizar Mahlaoui, Danielle Canioni, Nicole Brousse, Jean-Philippe Jais, Alain Fischer, and Olivier Hermine, H\u00f4pital Universitaire Necker\u2013Enfants Malades, Assistance Publique\u2013H\u00f4pitaux de Paris; Felipe Suarez, Nizar Mahlaoui, Chantal Andriamanga, Alain Fischer, and Olivier Hermine, French National Reference Center for Primary Immune Deficiency; Felipe Suarez, Nizar Mahlaoui, Jean-Philippe Jais, Alain Fischer, and Olivier Hermine, Imagine Institute, Institut National de la Recherche..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Hermine","sequence":"additional","affiliation":[{"name":"Felipe Suarez, Nizar Mahlaoui, Danielle Canioni, Nicole Brousse, Jean-Philippe Jais, Alain Fischer, and Olivier Hermine, H\u00f4pital Universitaire Necker\u2013Enfants Malades, Assistance Publique\u2013H\u00f4pitaux de Paris; Felipe Suarez, Nizar Mahlaoui, Chantal Andriamanga, Alain Fischer, and Olivier Hermine, French National Reference Center for Primary Immune Deficiency; Felipe Suarez, Nizar Mahlaoui, Jean-Philippe Jais, Alain Fischer, and Olivier Hermine, Imagine Institute, Institut National de la Recherche..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominique","family":"Stoppa-Lyonnet","sequence":"additional","affiliation":[{"name":"Felipe Suarez, Nizar Mahlaoui, Danielle Canioni, Nicole Brousse, Jean-Philippe Jais, Alain Fischer, and Olivier Hermine, H\u00f4pital Universitaire Necker\u2013Enfants Malades, Assistance Publique\u2013H\u00f4pitaux de Paris; Felipe Suarez, Nizar Mahlaoui, Chantal Andriamanga, Alain Fischer, and Olivier Hermine, French National Reference Center for Primary Immune Deficiency; Felipe Suarez, Nizar Mahlaoui, Jean-Philippe Jais, Alain Fischer, and Olivier Hermine, Imagine Institute, Institut National de la Recherche..."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"233","reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1159\/000106149"},{"key":"B2","doi-asserted-by":"crossref","first-page":"526","DOI":"10.1542\/peds.21.4.526","volume":"21","author":"Boder E","year":"1958","journal-title":"Pediatrics"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-pathol-011811-132509"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1126\/science.7792600"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1038\/nrm3546"},{"key":"B6","first-page":"1849","volume":"61","author":"Kamsler A","year":"2001","journal-title":"Cancer Res"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-08-373639"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(64)91209-7"},{"key":"B9","first-page":"89","volume":"77","author":"Morrell D","year":"1986","journal-title":"J Natl Cancer Inst"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V52.5.1003.1003"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V73.5.1285.1285"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V74.6.2076.2076"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.2870050407"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1038\/nm1097-1155"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V91.10.3920.3920_3920_3926"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.1999.01257.x"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2011.41.0852"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V87.2.423.bloodjournal872423"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-911X(199812)31:6<491::AID-MPO5>3.0.CO;2-B"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.1002\/mpo.10251"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199112263252602"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2011.08863.x"},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2011.03.052"},{"key":"B24","doi-asserted-by":"publisher","DOI":"10.1038\/bjc.2011.266"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2010.02.021"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.1214\/aos\/1176350951"},{"key":"B27","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2249.2008.03684.x"},{"key":"B28","doi-asserted-by":"publisher","DOI":"10.1158\/0008-5472.CAN-13-0022"},{"key":"B29","doi-asserted-by":"publisher","DOI":"10.1128\/JVI.00917-12"},{"key":"B30","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.24696"}],"container-title":["Journal of Clinical Oncology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/ascopubs.org\/doi\/pdfdirect\/10.1200\/JCO.2014.56.5101","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,5,25]],"date-time":"2022-05-25T19:44:59Z","timestamp":1653507899000},"score":1,"resource":{"primary":{"URL":"https:\/\/ascopubs.org\/doi\/10.1200\/JCO.2014.56.5101"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,1,10]]},"references-count":30,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2015,1,10]]}},"alternative-id":["10.1200\/JCO.2014.56.5101"],"URL":"https:\/\/doi.org\/10.1200\/jco.2014.56.5101","relation":{},"ISSN":["0732-183X","1527-7755"],"issn-type":[{"value":"0732-183X","type":"print"},{"value":"1527-7755","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,1,10]]},"assertion":[{"value":"2014-12-08","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}