Tγδ LGL LEUKEMIA IDENTIFIES A SUBSET WITH MORE SYMPTOMATIC DISEASE: ANALYSIS OF AN INTERNATIONAL COHORT OF 137 PATIENTS - Université de Rennes Accéder directement au contenu
Article Dans Une Revue Blood Année : 2023

Tγδ LGL LEUKEMIA IDENTIFIES A SUBSET WITH MORE SYMPTOMATIC DISEASE: ANALYSIS OF AN INTERNATIONAL COHORT OF 137 PATIENTS

Heejin Cheon
Antonella Teramo
Giulia Calabretto
  • Fonction : Auteur
Jasmanet Chahal
  • Fonction : Auteur
Cristina Vicenzetto
Julia Almeida
Bryna C Shemo
  • Fonction : Auteur
Min Shi
  • Fonction : Auteur
Vanessa Rebecca Gasparini
  • Fonction : Auteur
Noemi Munoz-Garcia
Hideyuki Nakazawa
Kazuo Oshimi
  • Fonction : Auteur
Lubomir Sokol
  • Fonction : Auteur
Fumihiro Ishida
  • Fonction : Auteur
Alberto Orfao
  • Fonction : Auteur
William G Morice
  • Fonction : Auteur
Thomas P Loughran
  • Fonction : Auteur

Résumé

Tγδ large granular lymphocyte leukemia (LGLL) is a rare variant of T-cell LGLL (T-LGLL) that has been less investigated as compared with the more frequent Tαβ LGLL, particularly in terms of frequency of STAT3 and STAT5b mutations. In this study, we characterized the clinical and biological features of 137 patients affected by Tγδ LGLL; data were retrospectively collected from 1997 to 2020 at 8 referral centers. Neutropenia and anemia were the most relevant clinical features, being present in 54.2% and 49.6% of cases, respectively, including severe neutropenia and anemia in ∼20% of cases each. Among the various treatments, cyclosporine A was shown to provide the best response rates. DNA samples of 97 and 94 cases were available for STAT3 and STAT5b mutation analysis, with 38.1% and 4.2% of cases being mutated, respectively. Clinical and biological features of our series of Tγδ cases were also compared with a recently published Tαβ cohort including 129 cases. Though no differences in STAT3 and STAT5b mutational frequency were found, Tγδ cases more frequently presented with neutropenia (P = .0161), anemia (P < .0001), severe anemia (P = .0065), and thrombocytopenia (P = .0187). Moreover, Vδ2- cases displayed higher frequency of symptomatic disease. Overall, Tγδ cases displayed reduced survival with respect to Tαβ cases (P = .0017). Although there was no difference in STAT3 mutation frequency, our results showed that Tγδ LGLL represents a subset of T-LGLL characterized by more frequent symptoms and reduced survival as compared with Tαβ LGLL.

Domaines

Cancer
Fichier principal
Vignette du fichier
Barilà et al-2022-Tγδ LGL LEUKEMIA IDENTIFIES A SUBSET WITH MORE SYMPTOMATIC DISEASE.pdf (1.13 Mo) Télécharger le fichier
1-s2.0-S000649712201206X-mmc1.pdf (1.63 Mo) Télécharger le fichier
Origine : Fichiers produits par l'(les) auteur(s)

Dates et versions

hal-03903415 , version 1 (16-12-2022)

Licence

Paternité - Pas de modifications

Identifiants

Citer

Gregorio Barilà, Angela Grassi, Heejin Cheon, Antonella Teramo, Giulia Calabretto, et al.. Tγδ LGL LEUKEMIA IDENTIFIES A SUBSET WITH MORE SYMPTOMATIC DISEASE: ANALYSIS OF AN INTERNATIONAL COHORT OF 137 PATIENTS. Blood, 2023, 141 (9), pp.1036-1046. ⟨10.1182/blood.2021013489⟩. ⟨hal-03903415⟩
28 Consultations
35 Téléchargements

Altmetric

Partager

Gmail Facebook X LinkedIn More