Imatinib is effective in some PDGFRA/B-negative hypereosinophilic syndromes: A step closer to unveiling underlying mechanisms.

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  • Author(s): Loscocco GG;Loscocco GG; Helbig G; Helbig G
  • Source:
    British journal of haematology [Br J Haematol] 2024 Dec; Vol. 205 (6), pp. 2136-2138. Date of Electronic Publication: 2024 Oct 28.
  • Publication Type:
    Journal Article
  • Language:
    English
  • Additional Information
    • Source:
      Publisher: Wiley-Blackwell Country of Publication: England NLM ID: 0372544 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1365-2141 (Electronic) Linking ISSN: 00071048 NLM ISO Abbreviation: Br J Haematol Subsets: MEDLINE
    • Publication Information:
      Publication: Oxford : Wiley-Blackwell
      Original Publication: Oxford : Blackwell Scientific Publications
    • Subject Terms:
      Hypereosinophilic Syndrome*/drug therapy ; Hypereosinophilic Syndrome*/genetics ; Imatinib Mesylate*/therapeutic use ; Receptor, Platelet-Derived Growth Factor alpha*/genetics; Humans ; Receptor, Platelet-Derived Growth Factor beta/genetics ; Male
    • Abstract:
      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; 205:2305-2314.
      (© 2024 The Author(s). British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd.)
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    • Contributed Indexing:
      Keywords: PDGFRA; PDGFRB; eosinophilia; hypereosinophilic syndrome; imatinib
    • Accession Number:
      8A1O1M485B (Imatinib Mesylate)
      EC 2.7.10.1 (Receptor, Platelet-Derived Growth Factor alpha)
      EC 2.7.10.1 (Receptor, Platelet-Derived Growth Factor beta)
      EC 2.7.10.1 (PDGFRB protein, human)
    • Publication Date:
      Date Created: 20241029 Date Completed: 20241212 Latest Revision: 20241214
    • Publication Date:
      20241214
    • Accession Number:
      PMC11637715
    • Accession Number:
      10.1111/bjh.19853
    • Accession Number:
      39468717