The high efficacy of tyrosine kinase inhibitor, imatinib mesylate (IM), is well-documented
in patients with hypereosinophilic syndromes (HES) and detectable rearrangements of
platelet-derived growth factor receptor α or β (PDGFRA/B) [
[1]
]. Most of these IM responders were found to possess an interstitial deletion in chromosome
4q12 leading to constitutively active tyrosine kinase, FIP1L1-PDGFRA (F/P) [
[2]
]. In contrast, the place of IM in the treatment of unmutated HES remains to be elucidated.
Of note is, that IM was found to be ineffective in most HES patients with unknown
or negative PDGFRA rearrangement. Among 18 treated patients, 1 complete and 2 partial
responses were achieved. Starting IM dose was 100 mg daily for 4 weeks and the dose could be escalated to 400 mg daily if there was no response or disease progression. IM was used as frontline
therapy in 7 patients [
[3]
]. Only a few unmutated HES patients responded to standard IM doses in two large prospective
studies, but response duration remained short in at least some of the treated cases
[
4
,
5
].Keywords
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Article info
Publication history
Published online: March 08, 2016
Accepted:
February 22,
2016
Received:
February 15,
2016
Identification
Copyright
© 2016 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
