Moving towards a uniform risk stratification system in CMML - How far are we?
CMML mutations
CMML prognostic scores
CMML risk assessment
Chronic myelomonocytic leukemia
Journal
Best practice & research. Clinical haematology
ISSN: 1532-1924
Titre abrégé: Best Pract Res Clin Haematol
Pays: Netherlands
ID NLM: 101120659
Informations de publication
Date de publication:
06 2020
06 2020
Historique:
received:
25
11
2019
revised:
29
11
2019
accepted:
02
12
2019
entrez:
29
5
2020
pubmed:
29
5
2020
medline:
1
7
2021
Statut:
ppublish
Résumé
Many prognostic scoring systems have been developed for chronic myelomonocytic leukemia (CMML). Although these efforts have been informative, no single model has been considered the consensus for CMML prognostication and all models are only moderately prognostic. CMML clinical models utilize mainly hematology and morphology parameters to estimate risk. A better understanding of cytogenetics and the genomic landscape of CMML have resulted in integrated risk models such as CMML Prognostic Scoring System (CPSS)-Mol and Mayo Molecular that may provide better prognostic accuracy for an individual patient. For example, frameshift/nonsense ASXL1 mutations have been consistently shown to confer inferior outcomes leading to its incorporation into some of the major risk classification systems. Prognostication in the setting of therapeutic interventions such as hypomethylating agents and allogeneic hematopoietic cell transplantation have also garnered considerable interest. Despite having many validated risk models available, not a single system is universally adopted. Herein, we will provide an overview of how these systems evolved and progress toward a uniform system.
Identifiants
pubmed: 32460982
pii: S1521-6926(19)30125-2
doi: 10.1016/j.beha.2019.101131
pii:
doi:
Substances chimiques
ASXL1 protein, human
0
Codon, Nonsense
0
Neoplasm Proteins
0
Repressor Proteins
0
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
101131Informations de copyright
Copyright © 2019 Elsevier Ltd. All rights reserved.
Déclaration de conflit d'intérêts
Declaration of competing interest The authors have no relevant conflicts of interest to disclose.