{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,11]],"date-time":"2026-10-11T07:57:57Z","timestamp":1791705477370,"version":"4.3.4"},"reference-count":137,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2022,4,14]],"date-time":"2022-04-14T00:00:00Z","timestamp":1649894400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000921","name":"European Cooperation in Science and Technology","doi-asserted-by":"publisher","award":["CA16223"],"award-info":[{"award-number":["CA16223"]}],"id":[{"id":"10.13039\/501100000921","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004442","name":"National Science Centre","doi-asserted-by":"publisher","award":["2017\/26\/D\/NZ5\/00811"],"award-info":[{"award-number":["2017\/26\/D\/NZ5\/00811"]}],"id":[{"id":"10.13039\/501100004442","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Patients with double stranded DNA repair disorders (DNARDs) (Ataxia Telangiectasia (AT) and Nijmegen Breakage syndrome (NBS)) are at a very high risk for developing hematological malignancies in the first two decades of life. The most common neoplasms are T-cell lymphoblastic malignancies (T-cell ALL and T-cell LBL) and diffuse large B cell lymphoma (DLBCL). Treatment of these patients is challenging due to severe complications of the repair disorder itself (e.g., congenital defects, progressive movement disorders, immunological disturbances and progressive lung disease) and excessive toxicity resulting from chemotherapeutic treatment. Frequent complications during treatment for malignancies are deterioration of pre-existing lung disease, neurological complications, severe mucositis, life threating infections and feeding difficulties leading to significant malnutrition. These complications make modifications to commonly used treatment protocols necessary in almost all patients. Considering the rarity of DNARDs it is difficult for individual physicians to obtain sufficient experience in treating these vulnerable patients. Therefore, a team of experts assembled all available knowledge and translated this information into best available evidence-based treatment recommendations.<\/jats:p>","DOI":"10.3390\/cancers14082000","type":"journal-article","created":{"date-parts":[[2022,4,14]],"date-time":"2022-04-14T21:44:06Z","timestamp":1649972646000},"page":"2000","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":30,"title":["Consensus Recommendations for the Clinical Management of Hematological Malignancies in Patients with DNA Double Stranded Break Disorders"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3089-6947","authenticated-orcid":false,"given":"Agata","family":"Pastorczak","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Oncology and Hematology, Medical University of Lodz, 91-738 Lodz, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9285-6898","authenticated-orcid":false,"given":"Andishe","family":"Attarbaschi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Pediatric Hematology and Oncology, St. Anna Children\u2019s Hospital, Medical University of Vienna, 1090 Vienna, Austria"},{"name":"Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, 1090 Vienna, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon","family":"Bomken","sequence":"additional","affiliation":[{"name":"Great North Children\u2019s Hospital, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne NE7 7DN, UK"},{"name":"Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne NE2 4HH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6121-4737","authenticated-orcid":false,"given":"Arndt","family":"Borkhardt","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology, Hematology and Clinical Immunology, University Children\u2019s Hospital, Medical Faculty, Heinrich Heine University, 40225 D\u00fcsseldorf, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jutte","family":"van der Werff ten Bosch","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology, Oncology and Immunology, University Hospital Brussels, 1090 Jette Brussels, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Elitzur","sequence":"additional","affiliation":[{"name":"Pediatric Hematology-Oncology, Schneider Children\u2019s Medical Center, Petach Tikvah 4920235, 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Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5336-261X","authenticated-orcid":false,"given":"Tomasz","family":"Szczepanski","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology and Oncology, Medical University of Silesia (SUM), 41-800 Zabrze, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tessa","family":"Wassenberg","sequence":"additional","affiliation":[{"name":"Department of Neurology and Child Neurology, Donders Institute for Brain, Cognition and Behavior, Radboud University Medical Center, 6525 GA Nijmegen, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Loeffen","sequence":"additional","affiliation":[{"name":"Princess M\u00e1xima Center for Pediatric Oncology, 3584 CS Utrecht, The 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