J 2023

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

PASTORCZAK, Agata, Bartosz SZMYD, Marcin BRAUN, Joanna MADZIO, Kamila WYPYSZCZAK et. al.

Basic information

Original name

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

Authors

PASTORCZAK, Agata (guarantor), Bartosz SZMYD, Marcin BRAUN, Joanna MADZIO, Kamila WYPYSZCZAK, Pawel SZTROMWASSER, Wojciech FENDLER, Marzena WOJTASZEWSKA, Jedrzej CHRZANOWSKI, Wieslawa GRAJKOWSKA, Hanna GREGOREK, Anna WAKULINSKA, Bernarda KAZANOWSKA, Zdenka KŘENOVÁ (203 Czech Republic, belonging to the institution), Dilys D WEIJERS, Roland P KUIPER and Wojciech MLYNARSKI

Edition

Haematologica, PAVIA, FERRATA STORTI FOUNDATION, 2023, 0390-6078

Other information

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30204 Oncology

Country of publisher

Italy

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

Impact factor

Impact factor: 10.100 in 2022

RIV identification code

RIV/00216224:14110/23:00133398

Organization unit

Faculty of Medicine

UT WoS

001109389400020

Keywords in English

large B-cell lymphomas; children

Tags

Tags

International impact, Reviewed
Změněno: 14/2/2024 09:43, Mgr. Tereza Miškechová

Abstract

V originále

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.

Links

MUNI/A/1395/2022, interní kód MU
Name: Personalizovaná léčba v dětské onkologii: multimodální theranostický přístup a „N-of-1 clinical trials“
Investor: Masaryk University