International Journal of Hematology

, Volume 89, Issue 1, pp 66–70

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

Authors

  • Chun-Yan Sun
    • Institute of Hematology, Union Hospital Tongji Medical CollegeHuazhong University of Science and Technology
    • Institute of Hematology, Union Hospital Tongji Medical CollegeHuazhong University of Science and Technology
  • Zhang-Bo Chu
    • Institute of Hematology, Union Hospital Tongji Medical CollegeHuazhong University of Science and Technology
  • Tao Guo
    • Institute of Hematology, Union Hospital Tongji Medical CollegeHuazhong University of Science and Technology
  • Jing He
    • Institute of Hematology, Union Hospital Tongji Medical CollegeHuazhong University of Science and Technology
Case Report

DOI: 10.1007/s12185-008-0205-1

Cite this article as:
Sun, C., Hu, Y., Chu, Z. et al. Int J Hematol (2009) 89: 66. doi:10.1007/s12185-008-0205-1

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.

Keywords

Hypereosinophilic syndromeFIP1-like-1-platelet-derived growth factor alpha (FIP1L1-PDGFRA)ImatinibValvular replacement

Supplementary material

12185_2008_205_MOESM1_ESM.doc (28 kb)
Reverse-transcriptase polymerase chain reaction (RT-PCR) analyses of FIP1L1-PDGFRA Fusion (DOC 27 kb)

Copyright information

© The Japanese Society of Hematology 2008