How I treat hypereosinophilic syndromes

Blood. 2015 Aug 27;126(9):1069-77. doi: 10.1182/blood-2014-11-551614. Epub 2015 May 11.


Hypereosinophilic syndromes (HESs) are a group of rare disorders characterized by peripheral blood eosinophilia of 1.5 × 10(9)/L or higher and evidence of end organ manifestations attributable to the eosinophilia and not otherwise explained in the clinical setting. HESs are pleomorphic in clinical presentation and can be idiopathic or associated with a variety of underlying conditions, including allergic, rheumatologic, infectious, and neoplastic disorders. Moreover, the etiology of the eosinophilia in HESs can be primary (myeloid), secondary (lymphocyte-driven), or unknown. Although corticosteroids remain the first-line therapy for most forms of HESs, the availability of an increasing number of novel therapeutic agents, including tyrosine kinase inhibitors and monoclonal antibodies, has necessarily altered the approach to treatment of HESs. This review presents an updated treatment-based approach to the classification of patients with presumed HES and discusses the roles of conventional and novel agents in the management of these patients.

Publication types

  • Case Reports
  • Research Support, N.I.H., Intramural
  • Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Adult
  • Antibodies, Monoclonal / therapeutic use
  • Drug Discovery
  • Female
  • Humans
  • Hypereosinophilic Syndrome / blood
  • Hypereosinophilic Syndrome / diagnosis
  • Hypereosinophilic Syndrome / drug therapy*
  • Imatinib Mesylate / therapeutic use
  • Protein Kinase Inhibitors / therapeutic use


  • Adrenal Cortex Hormones
  • Antibodies, Monoclonal
  • Protein Kinase Inhibitors
  • Imatinib Mesylate