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Hypereosinophilic Syndrome

  • Chapter
Myeloproliferative Disorders

Part of the book series: Hematologic Malignancies ((HEMATOLOGIC))

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Abstract

Hypereosinophilic syndrome (HES) is characterized by persistent overproduction of eosinophils, and the exclusion of other known causes of eosinophilia. Clinical manifestations of HES are related to eosinophilic infiltration of end organs that may include the skin, heart, lung, central nervous system, and gastrointestinal tract. Treatment has been largely empirically derived, and may include steroids, cytotoxic agents, and immunomodulatory agents. It was recently observed that a subset of patients diagnosed with HES demonstrated remarkable clinical responses to empiric treatment with the small molecule tyrosine kinase inhibitor imatinib. These responses suggested that an imatinib-sensitive tyrosine kinase contributed to pathogenesis of HES in these cases, and led to the cloning of FIP1L1-PDGFRA. FIP1L1-PPDGFRA is a constitutively activated tyrosine kinase that is imatinib sensitive, and is expressed as a consequence of an interstitial chromosomal deletion on chromosome 4. In addition to imatinib, other targeted therapies have evolved for treatment of HES, including monoclonal antibody therapy directed against IL-5. Thus, significant progress has been made in our understanding of the pathogenesis and therapy of HES in recent years, and has had an impact on approach to classification and clinical management.

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Authors and Affiliations

  1. Division of Hematology, Department of Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, 02115, USA

    Elizabeth H. Stover

  2. Howard Hughes Medical Institute, Boston, MA, 02115, USA

    Elizabeth H. Stover

  3. Stanford University School of Medicine, Stanford, CA, 94305, USA

    Jason Gotlib

  4. Department of Human Genetics, University of Leuven, Leuven, Belgium

    Jan Cools

  5. Karp Family Research Laboratories, Howard Hughes Medical Institute, Harvard Medical School, 1 Blackfan Circle, Room 5210, Boston, MA, 02115, USA

    D. Gary Gilliland

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  1. Elizabeth H. Stover
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  4. D. Gary Gilliland
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Stover, E.H., Gotlib, J., Cools, J., Gilliland, D.G. (2007). Hypereosinophilic Syndrome. In: Myeloproliferative Disorders. Hematologic Malignancies. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-34506-0_14

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