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Hypereosinophilic Syndrome and Eosinophilic Gastrointestinal Disorders Tomohiko Moriyama 1 , Motohiro Esaki 1 , Koji Kato 2 , Katsuto Takenaka 3 , Risa Ichinose 4 , Yoshiteru Kumagae 4 , Minako Hirahashi 4 , Takayuki Matsumoto 1 1Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan 2Department of Medicine and Biosystemic Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan 3Center for Cellular and Molecular Medicine, Kyushu University Hospital, Fukuoka, Japan 4Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan Keyword: 好酸球増多症候群 , 好酸球性消化管疾患 , FIP1L1-PDGFRA融合遺伝子 pp.1921-1929
Published Date 2013/12/25
DOI https://doi.org/10.11477/mf.1403114024
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 HES(hypereosinophilic syndrome)should be considered in the differential diagnosis of EGID(eosinophilic gastrointestinal disorders). After clarifying the mechanism of eosinophilia in HES, excluding other diseases with eosinophilia, identification of FIP1L1-PDGFRA fusion, and bone marrow examination become necessary in diagnosing HES. In patients with FIP1L1-PDGFRA fusion positive HES, imatinib mesylate leads to dramatic response. Corticosteroids, hydroxyurea, and interferon-α are effective in patients with FIP1L1-PDGFRA fusion negative HES. Genetic screening, functional analysis of lymphocytes, and evaluation for organ damage, including the cardiovascular and neurologic systems are needed for EGID patients with hypereosinophilia, steroid-dependence, and steroid-resistance.


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電子版ISSN 1882-1219 印刷版ISSN 0536-2180 医学書院

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