Revisiting Richter transformation in the era of novel CLL agents.
Chronic lymphocytic leukaemia
Genetics
Ibrutinib
Richter's transformation
TP53 disruption
Venetoclax
Journal
Blood reviews
ISSN: 1532-1681
Titre abrégé: Blood Rev
Pays: England
ID NLM: 8708558
Informations de publication
Date de publication:
09 2021
09 2021
Historique:
received:
04
08
2020
revised:
14
01
2021
accepted:
19
02
2021
pubmed:
30
3
2021
medline:
18
11
2021
entrez:
29
3
2021
Statut:
ppublish
Résumé
Richter transformation (RT) is the development of aggressive lymphoma - most frequently diffuse large B-cell lymphoma (DLBCL) and rarely Hodgkin lymphoma (HL) - arising on the background of chronic lymphocytic leukaemia (CLL). Despite recent advances in CLL treatment, RT also develops in patients on novel agents, usually occurring as an early event. RT incidence is lower in CLL patients treated with novel agents in the front line compared to relapsed/refractory cases, with a higher incidence in patients with TP53 disruption. The genetic heterogeneity and complexity are higher in RT-DLBCL than CLL; the genetics of RT-HL are largely unknown. In addition to TP53, aberrations in CDKN2A, MYC, and NOTCH1 are common in RT-DLBCL; however, no distinct RT-specific genetic aberration is recognised yet. RT-DLBCL on ibrutinib is frequently associated with BTK and PLCG2 mutations. Here, we update on genetic analysis, diagnostics and treatment options in RT in the era of novel agents.
Identifiants
pubmed: 33775465
pii: S0268-960X(21)00030-8
doi: 10.1016/j.blre.2021.100824
pii:
doi:
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
100824Informations de copyright
Copyright © 2021 The Authors. Published by Elsevier Ltd.. All rights reserved.