Lymphocytic variant hypereosinophilic syndromes

Immunol Allergy Clin North Am. 2007 Aug;27(3):389-413. doi: 10.1016/j.iac.2007.07.002.

Abstract

A large body of evidence establishing the existence of an underlying T-cell disorder in a subset of patients fulfilling hypereosinophilic syndrome (HES) diagnostic criteria has accumulated over the past decade, resulting in the definition of a novel HES variant termed "lymphocytic" HES. Although end-organ complications of hypereosinophilia are generally benign, with predominant cutaneous manifestations, long-term prognosis is overshadowed by an increased risk of developing T-cell lymphoma, as a result of malignant transformation of aberrant T cells years after HES diagnosis. Therapeutic strategies should target pathogenic T cells in addition to eosinophils, but the practical implications remain largely unexplored.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Antigens, CD / metabolism
  • Cytokines / metabolism*
  • Humans
  • Hypereosinophilic Syndrome* / diagnosis
  • Hypereosinophilic Syndrome* / immunology
  • Hypereosinophilic Syndrome* / physiopathology
  • Hypereosinophilic Syndrome* / therapy
  • Myeloproliferative Disorders / physiopathology
  • T-Lymphocyte Subsets / physiology*

Substances

  • Antigens, CD
  • Cytokines