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Successful treatment of myeloid neoplasms associated with PDGFRA rearrangement with imatinib mesylate

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Abstract

Hypereosinophilic syndromes (HES) constitute a rare and heterogeneous group of disorders, defined as persistent and marked blood eosinophilia associated with evidence of eosinophil-induced organ damage. Cardiac dysfunction is the most frequent result of end-organ damage and is the major cause of morbidity and mortality among patients with HES. Despite patients with FIP1-like-1-platelet-derived growth factor alpha (FIP1L1-PDGFRA) associated HES (myeloid neoplasms associated with PDGFRA rearrangement) have been shown to respond to low-dose imatinib with a complete and durable hematological and cytogenetic remission, influences of imatinib on clinical manifestations related to hypereosinophilia heart involvement are variable. Here we describe the case of a young male patient with severe heart involvement who had a prompt, clinical and hematological complete remission following administration of imatinib. However, as endomyocardial fibrosis and related loss of function are deteriorated after initiation of imatinib therapy, valvular replacement and tricuspid annuloplasty had to perform to restore his heart function. Our finding concurs with recent reports that severe heart involvement was irreversible with imatinib treatment.

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References

  1. Weller PF, Bubley GJ. The idiopathic hypereosinophilic syndrome. Blood. 1994;83:2759–79.

    CAS  PubMed  Google Scholar 

  2. Assa’ad AH, Spicer RL, Nelson DP, Zimmermann N, Rothenberg ME. Hypereosinophilic syndromes. Chem Immunol. 2000;76:208–29. doi:10.1159/000058787.

    Article  PubMed  Google Scholar 

  3. Fauci AS, Harley JB, Roberts WC, Ferrans VJ, Gralnick HR, Bjornson BH. NIH conference: the idiopathic hypereosinophilic syndrome—clinical, pathophysiologic, and therapeutic considerations. Ann Intern Med. 1982;97:78–92.

    Article  CAS  PubMed  Google Scholar 

  4. Ault P, Cortes J, Koller C, Kaled E, Kantarjian H. Response of idiopathic hypereosinophilic syndrome to treatment with imatinib mesylate. Leuk Res. 2002;26:881–4. doi:10.1016/S0145-2126(02)00046-2.

    Article  CAS  PubMed  Google Scholar 

  5. Wilkins HJ, Crane MM, Copeland K, Williams WV. Hypereosinophilic syndrome: an update. Am J Hematol. 2005;80:148–57. doi:10.1002/ajh.20423.

    Article  PubMed  Google Scholar 

  6. Tefferi A, Vardiman JW. Classification and diagnosis of myeloproliferative neoplasms: the 2008 World Health Organization criteria and point-of-care diagnostic algorithms. Leukemia. 2008;22:14–22. doi:10.1038/sj.leu.2404955.

    Article  CAS  PubMed  Google Scholar 

  7. Cools J, Stover EH, Gilliland DG. Detection of the FIP1L1-PDGFRA fusion in idiopathic hypereosinophilic syndrome and chronic eosinophilic leukemia. Methods Mol Med. 2006;125:177–87.

    CAS  PubMed  Google Scholar 

  8. Chusid MJ, Dale DC, West BC, Wolff SM. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore). 1975;54:1–27. doi:10.1097/00005792-197501000-00001.

    Article  CAS  Google Scholar 

  9. Gotlib J, Cools J, Malone JM, Schrier SL, Gilliland DG, Coutre SE. The FIP1L1-PDGFRα fusion tyrosine kinase in hypereosinophilic syndrome and chronic eosinophilic leukemia: implications for diagnosis, classification, and management. Blood. 2004;103:2879–91. doi:10.1182/blood-2003-06-1824.

    Article  CAS  PubMed  Google Scholar 

  10. Kalac M, Quintás-Cardama A, Vrhovac R, Kantarjian H, Verstovsek S. A critical appraisal of conventional and investigational drug therapy in patients with hypereosinophilic syndrome and clonal eosinophilia. Cancer. 2007;110(5):955–64. doi:10.1002/cncr.22920.

    Article  CAS  PubMed  Google Scholar 

  11. Roufosse FE, Goldman M, Cogan E. Hypereosinophilic syndromes. Orphanet J Rare Dis. 2007;2:37–48. doi:10.1186/1750-1172-2-37.

    Article  PubMed  PubMed Central  Google Scholar 

  12. Cools J, DeAngelo DJ, Gotlib J, et al. A tyrosine kinase created by fusion of the PDGFRA and FIPL1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med. 2003;348:1201–14. doi:10.1056/NEJMoa025217.

    Article  CAS  PubMed  Google Scholar 

  13. Cortes J, Ault P, Koller C, et al. Efficacy of imatinib mesylate in the treatment of idiopathic hypereosinophilic syndrome. Blood. 2003;101:4714–6. doi:10.1182/blood-2003-01-0081.

    Article  CAS  PubMed  Google Scholar 

  14. Cervetti G, Galimberti S, Carulli G, Petrini M. Imatinib therapy in hypereosinophilic syndrome: a case of molecular remission. Leuk Res. 2005;29:1097–8. doi:10.1016/j.leukres.2005.02.014.

    Article  PubMed  Google Scholar 

  15. Pardanani A, Ketterling RP, Li CY, et al. FIP1L1-PDGFRA in eosinophilic disorders: prevalence in routine clinical practice, long-term experience with imatinib therapy, and a critical review of the literature. Leuk Res. 2006;30:965–70. doi:10.1016/j.leukres.2005.11.011.

    Article  CAS  PubMed  Google Scholar 

  16. Baccarani M, Cilloni D, Rondoni M, et al. The efficacy of imatinib mesylate in patients with FIP1l1-PDGFRalpha-positive hypereosinophilic syndrome: results of a multicenter prospective study. Haematologica. 2007;92:1173–9. doi:10.3324/haematol.11420.

    Article  CAS  PubMed  Google Scholar 

  17. Anghel G, De Rosa L, Ruscio C, et al. Efficacy of imatinib mesylate in a patient with idiopathic hypereosinophilic syndrome and severe heart involvement. Tumori. 2005;91:67–70.

    PubMed  Google Scholar 

  18. Onitilo AA, Kio EA, Singh AK, Lazarchick J. Cytogenetic remission with imatinib therapy in hypereosinophilic syndrome with trisomy 8 and resolution of severe cardiac dysfunction. Leuk Lymphoma. 2005;46:1667–70. doi:10.1080/10428190500220514.

    Article  PubMed  Google Scholar 

  19. Klion AD, Robyn J, Akin C, et al. Molecular remission and reversal of myelofibrosis in response to imatinib mesylate treatment in patients with the myeloproliferative variant of hypereosinophilic syndrome. Blood. 2004;103:473–8. doi:10.1182/blood-2003-08-2798.

    Article  CAS  PubMed  Google Scholar 

  20. Vandenberghe P, Wlodarska I, Michaux L, et al. Clinical and molecular features of FIP1L1-PDFGRA (+) chronic eosinophilic leukemias. Leukemia. 2004;18:734–42. doi:10.1038/sj.leu.2403313.

    Article  CAS  PubMed  Google Scholar 

  21. Klion AD, Noel P, Akin C, et al. Elevated serum tryptase levels identify a subset of patients with a myeloproliferative variant of idiopathic hypereosinophilic syndrome associated with tissue fibrosis, poor prognosis, and imatinib responsiveness. Blood. 2003;101:4660–6. doi:10.1182/blood-2003-01-0006.

    Article  CAS  PubMed  Google Scholar 

  22. Jovanovic JV, Score J, Waghorn K, et al. Low-dose imatinib mesylate leads to rapid induction of major molecular responses and achievement of complete molecular remission in FIP1L1-PDGFRA-positive chronic eosinophilic leukemia. Blood. 2007;109:4635–40. doi:10.1182/blood-2006-10-050054.

    Article  CAS  PubMed  Google Scholar 

  23. Klion AD, Robyn J, Maric I, et al. Relapse following discontinuation of imatinib mesylate therapy for FIP1L1/PDGFRA-positive chronic eosinophilic leukemia: implications for optimal dosing. Blood. 2007;110:3552–6. doi:10.1182/blood-2007-07-100164.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

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Authors and Affiliations

  1. Institute of Hematology, Union Hospital Tongji Medical College, Huazhong University of Science and Technology, 430022, Wuhan, China

    Chun-Yan Sun, Yu Hu, Zhang-Bo Chu, Tao Guo & Jing He

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  1. Chun-Yan Sun
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  2. Yu Hu
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  3. Zhang-Bo Chu
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  4. Tao Guo
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  5. Jing He
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Correspondence to Yu Hu.

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Sun, CY., Hu, Y., Chu, ZB. et al. Successful treatment of myeloid neoplasms associated with PDGFRA rearrangement with imatinib mesylate. Int J Hematol 89, 66–70 (2009). https://doi.org/10.1007/s12185-008-0205-1

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  • DOI: https://doi.org/10.1007/s12185-008-0205-1

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