Consensus Recommendations for the Clinical Management of Hematological Malignancies in Patients with DNA Double Stranded Break Disorders

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 AL...

Full description

Saved in:
Bibliographic Details
Published in:Cancers 2022-04, Vol.14 (8), p.2000
Main Authors: Pastorczak, Agata, Attarbaschi, Andishe, Bomken, Simon, Borkhardt, Arndt, van der Werff Ten Bosch, Jutte, Elitzur, Sarah, Gennery, Andrew R, Hlavackova, Eva, Kerekes, Arpád, Křenová, Zdenka, Mlynarski, Wojciech, Szczepanski, Tomasz, Wassenberg, Tessa, Loeffen, Jan
Format: Article
Language:English
Subjects:
Ataxia
Ataxia telangiectasia
B-cell lymphoma
Cancer
Cell cycle
Chromosomes
Congenital defects
Congenital diseases
Cysts
Deoxyribonucleic acid
Disease
DNA
DNA damage
DNA repair
Ewings sarcoma
Genotype & phenotype
Hematology
Immune system
Immunoproliferative diseases
Intellectual disabilities
Kinases
Leukemia
Lung diseases
Lymphocytes
Lymphocytes T
Lymphoma
Malnutrition
Microcephaly
Movement disorders
Mucositis
Mutation
Neurological complications
Nijmegen breakage syndrome
Pathophysiology
Patients
Pediatrics
Proteins
Review
Thyroid gland
Toxicity
Transplants & implants
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:2072-6694
2072-6694
DOI:10.3390/cancers14082000