Allergy, Asthma & Respiratory Disease

The Korean Academy of Asthma, Allergy and Clinical Immunology (대한천식알레르기학회)
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Myeloid and lymphoid neoplasm with eosinophilia and abnormalities of PDGFRB presenting as congestive heart failure and hypereosinophilia

심부전과 과다호산구증가증으로 내원한 PDGFRB 유전변이가 동반된 골수성 종양

  • Kwon, Jae-Woo (Department of Allergy and Clinical Immunology, Kangwon National University School of Medicine) ;
  • Kwon, Ji-Hyun (Department of Internal Medicine, Chungbuk National College of Medicine) ;
  • Her, Ae-Young (Division of Cardiology, Department of Internal Medicine, Kangwon National University School of Medicine)
  • 권재우 (강원대학교 의학전문대학원 알레르기내과학교실) ;
  • 권지현 (충북대학교 의과대학 내과학교실) ;
  • 허애영 (강원대학교 의학전문대학원 심장내과학교실)
  • Published : 20170000
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Abstract

Hypereosinophilic syndrome (HES) is a heterogeneous disorder characterized by persistent hypereosinophilia with the evidence of organ dysfunction caused by eosinophilic involvement. HES can be induced by various secondary causes, including helminthic infections, adverse drug reactions, and allergic diseases. Primary/clonal bone marrow disease, including genetic mutations in platelet driven growth factor receptor alpha (PDGFRA), platelet driven growth factor receptor beta (PDGFRB), and fibroblast growth factor receptor 1 (FGFR1) could be its causes. Although corticosteroids are the mainstay of therapy in confirmed HES, imatinib is considered a definitive treatment for HES with these mutations. However, there have been few reports about HES with these genetic mutations in Korea. Here, we report a patient who presented with sudden onset of congestive heart failure and hypereosinophilia, proved to have PDGFRB rearrangement, and was controlled successfully with imatinib after left ventricle thrombectomy.

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