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

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00159816%3A_____%2F22%3A00076089" target="_blank" >RIV/00159816:_____/22:00076089 - isvavai.cz</a>

Alternative codes found

RIV/65269705:_____/22:00076089 RIV/00216224:14110/22:00128354

Result on the web

<a href="https://www.mdpi.com/2072-6694/14/8/2000" target="_blank" >https://www.mdpi.com/2072-6694/14/8/2000</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.3390/cancers14082000" target="_blank" >10.3390/cancers14082000</a>

Result language

angličtina

Original language name

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

Original language description

Simple Summary Ataxia Telangiectasia (AT) and Nijmegen breakage syndrome (NBS) are the most common DNA repair disorders (DNARDs), characterized by an exceedingly high risk for developing hematological malignancies and poor outcomes. Clinical management of lymphoproliferative diseases in AT and NBS is complicated due to the competing challenges of delivery of optimal cancer treatment and management of excessive toxicities. AT and NBS are rare genetic entities in the general population, thus gaining extensive experience in treatment of these patients is difficult. Additionally, no treatment guidelines for lymphoproliferative diseases have been specifically designed for this group of patients as yet. In this review we formulate clinical recommendations, considering the most critical aspects related to the management of lymphoproliferative disorders in AT and NBS and we concisely present the current state of knowledge about the biology and outcomes of leukemia and lymphoma in these DNARDs. 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.

Czech name

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Czech description

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Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

—

OECD FORD branch

30204 - Oncology

Project

—

Continuities

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year

2022

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Cancers

ISSN

2072-6694

e-ISSN

2072-6694

Volume of the periodical

14

Issue of the periodical within the volume

8

Country of publishing house

CH - SWITZERLAND

Number of pages

23

Pages from-to

2000

UT code for WoS article

000786061500001

EID of the result in the Scopus database

2-s2.0-85128242556