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

Kód výsledku v IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F65269705%3A_____%2F23%3A00078430" target="_blank" >RIV/65269705:_____/23:00078430 - isvavai.cz</a>

Nalezeny alternativní kódy

RIV/00216224:14110/23:00133398

Výsledek na webu

<a href="https://haematologica.org/article/view/haematol.2022.282325" target="_blank" >https://haematologica.org/article/view/haematol.2022.282325</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.3324/haematol.2022.282325" target="_blank" >10.3324/haematol.2022.282325</a>

Jazyk výsledku

angličtina

Název v původním jazyce

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

Popis výsledku v původním jazyce

Nijmegen breakage syndrome (NBS) is an inherited DNA repair disorder characterized by a high predisposition to develop lymphoid malignancies during childhood, with diffuse large B-cell lymphoma (DLBCL) being one of the leading types. Due to concomitant immunodeficiency and an increased risk of chemotherapy-induced toxicity, NBS patients often require modified and individualized cancer treatment. Nevertheless, they rarely achieve progressionand relapse-free long-term survival rates without hematopoietic stem cell transplantation. Unfavorable outcome of lymphomas in NBS may result not only from the reduction of drug dosages but could be associated with molecular aberrations occurring on the background of chromosomal instability. Therefore, we aimed to investigate clinical outcome, histopathological features, and genomic alterations of DLBCL in pediatric patients with NBS. The algorithm of the study is shown in the Online Supplementary Figure S1A.

Název v anglickém jazyce

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

Popis výsledku anglicky

Nijmegen breakage syndrome (NBS) is an inherited DNA repair disorder characterized by a high predisposition to develop lymphoid malignancies during childhood, with diffuse large B-cell lymphoma (DLBCL) being one of the leading types. Due to concomitant immunodeficiency and an increased risk of chemotherapy-induced toxicity, NBS patients often require modified and individualized cancer treatment. Nevertheless, they rarely achieve progressionand relapse-free long-term survival rates without hematopoietic stem cell transplantation. Unfavorable outcome of lymphomas in NBS may result not only from the reduction of drug dosages but could be associated with molecular aberrations occurring on the background of chromosomal instability. Therefore, we aimed to investigate clinical outcome, histopathological features, and genomic alterations of DLBCL in pediatric patients with NBS. The algorithm of the study is shown in the Online Supplementary Figure S1A.

Druh

J_imp - Článek v periodiku v databázi Web of Science

CEP obor

—

OECD FORD obor

30205 - Hematology

Projekt

—

Návaznosti

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Rok uplatnění

2023

Kód důvěrnosti údajů

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

Název periodika

Haematologica

ISSN

0390-6078

e-ISSN

1592-8721

Svazek periodika

108

Číslo periodika v rámci svazku

10

Stát vydavatele periodika

IT - Italská republika

Počet stran výsledku

6

Strana od-do

2808-2813

Kód UT WoS článku

001109389400020

EID výsledku v databázi Scopus

2-s2.0-85173573650