Clonal hematopoiesis and bone marrow failure syndromes.
Bone marrow failure
Clonal hematopoiesis
Leukemic transformation
Somatic reversion
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 2021
06 2021
Historique:
received:
03
05
2021
accepted:
11
05
2021
entrez:
18
8
2021
pubmed:
19
8
2021
medline:
14
1
2022
Statut:
ppublish
Résumé
Bone marrow failure syndromes (BMF) are a group of conditions characterized by inefficient hematopoiesis frequently associated with extra-hematopoietic phenotypes and variable risk of progression to myeloid malignancies. They can be acquired or inherited and mediated by either cell extrinsic factors or cell intrinsic impairment of hematopoietic stem cell (HSC) function. The pathophysiology includes immune-mediated attack (e.g., acquired BMFs) or germline defects in DNA damage repair machinery, telomeres maintenance or ribosomes biogenesis. (e.g., inherited BMF). Clonal hematopoiesis (CH) that frequently accompanies BMF may provide a mechanism of improved HSC fitness through the evasion of extracellular pressure or somatic reversion of germline defects. The mechanism for the CH selective advantage differs depending on the condition in which it occurs. However, this adaptation mechanism, particularly when involving putative oncogenes or tumor suppressors, may lead to increased risk of myeloid malignancies. Surveillance and early detection of leukemogenic clones may lead to timely implementation of curative therapies and improved survival.
Identifiants
pubmed: 34404525
pii: S1521-6926(21)00038-4
doi: 10.1016/j.beha.2021.101273
pmc: PMC8374084
mid: NIHMS1709711
pii:
doi:
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
101273Subventions
Organisme : NHLBI NIH HHS
ID : K08 HL136894
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL156144
Pays : United States
Informations de copyright
Copyright © 2021 Elsevier Ltd. All rights reserved.
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