Clinical features predict responsiveness to imatinib in platelet-derived growth factor receptor-alpha-negative hypereosinophilic syndrome

Allergy
P KhouryAmy D Klion

Abstract

With the exception of the presence of the FIP1L1-PDGFRA fusion gene, little is known about predictors of imatinib response in clinically-defined hypereosinophilic syndrome (HES). Subjects with FIP1L1-PDGFRA-myeloid neoplasm (FP; n =12), PDGFRA-negative HES with ≥4 criteria suggestive of a myeloid neoplasm (MHES; n =10), or steroid-refractory PDGFRA-negative HES with <4 myeloid criteria (SR; n = 5) were enrolled in a prospective study of imatinib therapy (NCT00044304: registered at clinicaltrials.gov). The primary outcome was an eosinophil count <1.5 × 109/L at one month and improvement of clinical symptoms. Clinical, molecular, and bone marrow responses to imatinib were assessed. A retrospective cohort of 18 subjects with clinically-defined HES who received imatinib (300-400 mg daily ≥ 1 month) were classified according to the criteria used in the prospective study. Overall, imatinib response rates were 100% in the FP group (n = 16), 54% in the MHES group (n = 13) and 0% in the SR group (n = 16). The presence of ≥ 4 myeloid features was the sole predictor of response. After ≥ 18 months in complete remission, imatinib was tapered and discontinued in 8 FP and 1 MHES subjects. Seven subjects (6 FP, 1 MHES) remain in remission off ...Continue Reading

References

Oct 28, 2008·British Journal of Haematology·Georgia MetzgerothAndreas Reiter
Nov 17, 2009·The Journal of Allergy and Clinical Immunology·Princess U OgboguAmy D Klion
Feb 18, 2011·Cancer Chemotherapy and Pharmacology·Grzegorz HelbigSławomira Kyrcz-Krzemień
Mar 31, 2012·The Journal of Allergy and Clinical Immunology·Peter ValentGerald J Gleich
Jul 3, 2013·Cytogenetic and Genome Research·A SimonsR J Hastings

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Citations

Mar 13, 2016·European Journal of Internal Medicine·Grzegorz Helbig
May 1, 2016·The Journal of Allergy and Clinical Immunology. in Practice·Kelli W WilliamsAmy D Klion
Jan 10, 2018·Frontiers in Medicine·Paneez KhouryAmy D Klion
Apr 4, 2019·The New England Journal of Medicine·Fei Li KuangAmy D Klion
Jan 25, 2020·Annual Review of Pathology·Amy D KlionBruce S Bochner
Apr 16, 2020·BMC Pulmonary Medicine·Jiaxing XieKefang Lai
Jan 24, 2017·British Journal of Haematology·Nauman M ButtUNKNOWN British Committee for Standards in Haematology
Jul 14, 2018·Clinical Reviews in Allergy & Immunology·Vivian C Nanagas, Anna Kovalszki
Apr 20, 2018·Journal of Leukocyte Biology·Paneez KhouryAmy D Klion
Apr 24, 2018·Current Hematologic Malignancy Reports·Melanie C Dispenza, Bruce S Bochner
Jul 7, 2018·Molecular and Clinical Oncology·Amanda Santos Dal BertoAntuani Rafael Baptistella
Jan 6, 2018·Expert Review of Clinical Immunology·Grzegorz Helbig
Oct 2, 2020·Chest·Chen E Rosenberg, Paneez Khoury
Oct 19, 2017·The Journal of Allergy and Clinical Immunology·Jeremy A O'Sullivan, Bruce S Bochner

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