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.

Department of Clinical Immunology and Allergology St Anne's University Hospital in Brno Faculty of Medicine Masaryk University 662 63 Brno Czech Republic

Department of Neurology and Child Neurology Donders Institute for Brain Cognition and Behavior Radboud University Medical Center 6525 GA Nijmegen The Netherlands

Department of Pediatric Hematology and Oncology Medical University of Silesia 41 800 Zabrze Poland

Department of Pediatric Hematology Oncology and Immunology University Hospital Brussels 1090 Jette Brussels Belgium

Department of Pediatric Oncology Hematology and Clinical Immunology University Children's Hospital Medical Faculty Heinrich Heine University 40225 Düsseldorf Germany

Department of Pediatric Oncology University Hospital and Faculty of Medicine Masaryk University 662 63 Brno Czech Republic

Department of Pediatrics and Adolescent Medicine Medical University of Vienna 1090 Vienna Austria

Department of Pediatrics Oncology and Hematology Medical University of Lodz 91 738 Lodz Poland

Department of Pediatrics Pediatric Hematology and Oncology St Anna Children's Hospital Medical University of Vienna 1090 Vienna Austria

Great North Children's Hospital Newcastle upon Tyne Hospitals NHS Foundation Trust Newcastle upon Tyne NE7 7DN UK

Pediatric Hematology Oncology Schneider Children's Medical Center Petach Tikvah 4920235 Israel

Princess Máxima Center for Pediatric Oncology 3584 CS Utrecht The Netherlands

Translational and Clinical Research Institute Newcastle University Newcastle upon Tyne NE2 4HH UK

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Overt and covert genetic causes of pediatric acute lymphoblastic leukemia

Clinical and laboratory diversity of diffuse large B-cell lymphomas in children with Nijmegen breakage syndrome