Defining TCRγδ lymphoproliferative disorders by combined immunophenotypic and molecular evaluation.
Journal
Nature communications
ISSN: 2041-1723
Titre abrégé: Nat Commun
Pays: England
ID NLM: 101528555
Informations de publication
Date de publication:
08 06 2022
08 06 2022
Historique:
received:
03
03
2021
accepted:
31
05
2022
entrez:
8
6
2022
pubmed:
9
6
2022
medline:
11
6
2022
Statut:
epublish
Résumé
Tγδ large granular lymphocyte leukemia (Tγδ LGLL) is a rare lymphoproliferative disease, scantily described in literature. A deep-analysis, in an initial cohort of 9 Tγδ LGLL compared to 23 healthy controls, shows that Tγδ LGLL dominant clonotypes are mainly public and exhibit different V-(D)-J γ/δ usage between patients with symptomatic and indolent Tγδ neoplasm. Moreover, some clonotypes share the same rearranged sequence. Data obtained in an enlarged cohort (n = 36) indicate the importance of a combined evaluation of immunophenotype and STAT mutational profile for the correct management of patients with Tγδ cell expansions. In fact, we observe an association between Vδ2/Vγ9 clonality and indolent course, while Vδ2/Vγ9 negativity correlates with symptomatic disease. Moreover, the 7 patients with STAT3 mutations have neutropenia and a CD56-/Vδ2- phenotype, and the 3 cases with STAT5B mutations display an asymptomatic clinical course and CD56/Vδ2 expression. All these data indicate that biological characterization is needed for Tγδ-cell neoplasm definition.
Identifiants
pubmed: 35676278
doi: 10.1038/s41467-022-31015-x
pii: 10.1038/s41467-022-31015-x
pmc: PMC9177852
doi:
Substances chimiques
Receptors, Antigen, T-Cell, gamma-delta
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
3298Informations de copyright
© 2022. The Author(s).
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