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Book

Hypereosinophilic Syndrome

In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan.
.
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Book

Hypereosinophilic Syndrome

Elizabeth S. Mikhail et al.
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Excerpt

Hypereosinophilic syndrome (HES) encompasses a rare and complex group of heterogeneous disorders characterized by persistent and substantial elevations in eosinophil levels and mediators leading to tissue infiltration and damage. Eosinophilopoiesis plays a vital role in the pro-inflammatory processes of multiple diseases.

The HES criteria are as follows:

  1. Hypereosinophilia is characterized by >1.5 x 109 eosinophils on CBC on 2 examinations >1 month apart.

  2. The percentage of eosinophils in the bone marrow (BM) section must exceed 20% of all nucleated cells

  3. The pathologist's assessment that the tissue infiltration by eosinophils is extensive and/or marked deposition of eosinophil granule proteins is found.

  4. Evidence of organ or tissue damage attributable to tissue hypereosinophilia (HE).

  5. Exclusion of other disorders or conditions as major reasons for organ damage.

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Conflict of interest statement

Disclosure: Elizabeth Mikhail declares no relevant financial relationships with ineligible companies.

Disclosure: Abhijeet Ghatol declares no relevant financial relationships with ineligible companies.

References

    1. Valent P, Klion AD, Horny HP, Roufosse F, Gotlib J, Weller PF, Hellmann A, Metzgeroth G, Leiferman KM, Arock M, Butterfield JH, Sperr WR, Sotlar K, Vandenberghe P, Haferlach T, Simon HU, Reiter A, Gleich GJ. Contemporary consensus proposal on criteria and classification of eosinophilic disorders and related syndromes. J Allergy Clin Immunol. 2012 Sep;130(3):607-612.e9. - PMC - PubMed
    1. Valent P, Klion AD, Roufosse F, Simon D, Metzgeroth G, Leiferman KM, Schwaab J, Butterfield JH, Sperr WR, Sotlar K, Vandenberghe P, Hoermann G, Haferlach T, Moriggl R, George TI, Akin C, Bochner BS, Gotlib J, Reiter A, Horny HP, Arock M, Simon HU, Gleich GJ. Proposed refined diagnostic criteria and classification of eosinophil disorders and related syndromes. Allergy. 2023 Jan;78(1):47-59. - PMC - PubMed
    1. Simon HU, Rothenberg ME, Bochner BS, Weller PF, Wardlaw AJ, Wechsler ME, Rosenwasser LJ, Roufosse F, Gleich GJ, Klion AD. Refining the definition of hypereosinophilic syndrome. J Allergy Clin Immunol. 2010 Jul;126(1):45-9. - PMC - PubMed
    1. Roufosse FE, Goldman M, Cogan E. Hypereosinophilic syndromes. Orphanet J Rare Dis. 2007 Sep 11;2:37. - PMC - PubMed
    1. Crane MM, Chang CM, Kobayashi MG, Weller PF. Incidence of myeloproliferative hypereosinophilic syndrome in the United States and an estimate of all hypereosinophilic syndrome incidence. J Allergy Clin Immunol. 2010 Jul;126(1):179-81. - PMC - PubMed

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