T cell large granular lymphocyte leukemia and chronic NK lymphocytosis.
LGLL
Neutropenia
STAT3
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:
09 2019
09 2019
Historique:
received:
10
04
2019
revised:
19
06
2019
accepted:
21
06
2019
entrez:
6
10
2019
pubmed:
6
10
2019
medline:
28
2
2020
Statut:
ppublish
Résumé
Large Granular Lymphocyte Leukemia (LGLL) is a rare chronic lymphoproliferative disorder characterized by the clonal expansion of Large Granular Lymphocytes (LGLs). Among LGLL, the 2016 WHO classification recognizes two different entities, i.e. T-LGLL and the provisional entity Chronic Lymphoproliferative disorder of NK cells (CLPD-NK). In both subtypes neutropenia represents the hallmark of the disease and is frequently regarded as the leading reason to start treatment. Leukemic LGLs are characterized by the up-regulation of several pro-survival signaling pathways, the most relevant being the JAK-STAT axis, whose constitutive activation is partly explained by somatic mutations in STAT3 and STAT5b. In addiction, in the last few years, a relationship between STAT3 mutations/activation and the development of neutropenia was found. Given that backbone treatment relying on immunosuppressive agents is generally unsatisfactory, novel agents targeting the JAK/STAT pathway can represent a turning point in LGLL treatment.
Identifiants
pubmed: 31585621
pii: S1521-6926(19)30039-8
doi: 10.1016/j.beha.2019.06.006
pii:
doi:
Substances chimiques
Neoplasm Proteins
0
STAT3 Transcription Factor
0
STAT3 protein, human
0
STAT5 Transcription Factor
0
STAT5B protein, human
0
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
207-216Informations de copyright
Copyright © 2019 Elsevier Ltd. All rights reserved.