Page 665 - Clinical Immunology_ Principles and Practice ( PDFDrive )

CHaPter 46 Eosinophil-Associated Gastrointestinal Disorders 639

in which a tyrosine kinase is overexpressed due to gene fusion. 22,24 HESs are a heterogeneous group of rare disorders, characterized
Notwithstanding the heterogeneity of underlying mechanisms, by persistent peripheral blood eosinophilia with more than 1500
3
the clinical manifestation of HES is similar, with eosinophil- cells/mm for longer than 6 months, no known cause of eosino-
mediated end organ tissue damage. philia, and signs and symptoms of organ involvement. Prognosis
depends on the degree and location of the eosinophil-mediated
Treatment end-organ tissue damage.
Like EGE, HES is a rare disorder, and no prospective treatment
studies have been carried out to date. Treatment strategies for Please check your eBook at https://expertconsult.inkling.com/
patients with HESs aim to lower the eosinophil tissue infiltration. for self-assessment questions. See inside cover for registration
Corticosteroids have been used for decades to treat HESs and details.
remain first-line therapy, except for PDGFRA-associated HESs.
Cytotoxic agents, such as hydroxyurea, vincristine, and chlor- REFERENCES
ambucil, have been successfully used in corticosteroid-refractory
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23
mucosal ulcerations. The tyrosine kinase inhibitor imatinib 2. Kato M, Kephart GM, Talley NJ, et al. Eosinophil infiltration and
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a valuable approach, given that patients with HESs often have updated consensus recommendations for children and adults. J Allergy
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• Clinical research is needed to:
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