Imatinib is effective in some PDGFRA/B-negative hypereosinophilic syndromes: A step closer to unveiling underlying mechanisms.
PDGFRA
PDGFRB
eosinophilia
hypereosinophilic syndrome
imatinib
Journal
British journal of haematology
ISSN: 1365-2141
Titre abrégé: Br J Haematol
Pays: England
ID NLM: 0372544
Informations de publication
Date de publication:
28 Oct 2024
28 Oct 2024
Historique:
received:
10
10
2024
accepted:
11
10
2024
medline:
29
10
2024
pubmed:
29
10
2024
entrez:
29
10
2024
Statut:
aheadofprint
Résumé
Hypereosinophilic syndromes (HES) comprise different clonal, reactive, or idiopathic disorders characterized by elevated eosinophil levels and subsequent organ damage. Kim et al. in a multicentre, single-arm, prospective phase II study, treated 32 patients with PDGFRA/B-negative HES with imatinib at the dose of 100-400 mg daily. Respective overall and complete haematological response rates were 46.9% and 18.8%, and the median time to response was 1.5 months. The molecular basis of responses was identified by using whole-exome and whole-transcriptome sequencing in 11 patients. STAT5B::RARA, PAK2::PIGX, and FIP1L1::CHIC2 fusions were identified in responders, whereas RNF130::BRAF and WNK1::KDM5A were identified in non-responders. Imatinib could be a therapeutic option for some, possibly clonal, PDGFRA/B-negative HES. Commentary on: Kim et al. Phase II trial of imatinib mesylate in patients with PDGFRA/B-negative hypereosinophilic syndrome. Br J Haematol 2024 (Online ahead of print). doi: 10.1111/bjh.19828.
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Informations de copyright
© 2024 The Author(s). British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd.
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