Pathogenesis and classification of eosinophil disorders: A review of recent developments in the field

Peter Valent*, Gerald J. Gleich, Andreas Reiter, Florence Roufosse, Peter F. Weller, Andrzej Hellmann, Georgia Metzgeroth, Kristin M. Leiferman, Michel Arock, Karl Sotlar, Joseph H. Butterfield, Sabine Cerny-Reiterer, Matthias Mayerhofer, Peter Vandenberghe, Torsten Haferlach, Bruce S. Bochner, Jason Gotlib, Hans Peter Horny, Hans Uwe Simon, Amy D. Klion

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

135 Scopus citations

Abstract

Eosinophils and their products play an essential role in the pathogenesis of various reactive and neoplastic disorders. Depending on the underlying disease, molecular defect and involved cytokines, hypereosinophilia may develop and may lead to organ damage. In other patients, persistent eosinophilia is accompanied by typical clinical findings, but the causative role and impact of eosinophilia remain uncertain. For patients with eosinophil-mediated organ pathology, early therapeutic intervention with agents reducing eosinophil counts can be effective in limiting or preventing irreversible organ damage. Therefore, it is important to approach eosinophil disorders and related syndromes early by using established criteria, to perform all appropriate staging investigations, and to search for molecular targets of therapy. In this article, we review current concepts in the pathogenesis and evolution of eosinophilia and eosinophil-related organ damage in neoplastic and non-neoplastic conditions. In addition, we discuss classifications of eosinophil disorders and related syndromes as well as diagnostic algorithms and standard treatment for various eosinophil-related disorders.

Original languageEnglish (US)
Pages (from-to)157-176
Number of pages20
JournalExpert Review of Hematology
Volume5
Issue number2
DOIs
StatePublished - 2012

Funding

P Valent has received a Research Grant from Novartis and a Research Grant from BMS, as well as honoraria from Novartis and BMS. AD Klein is supported by the Intramural Program of the NIAID, NIH. KM Leiferman, HU Simon and GJ Gleich have been supported by grants from GSK. M Arock received honorarium from Novartis. P Vandenberghe is a Senior Clinical Investigator of FWO‑Vlaanderen. T Haferlach is part owner of the Munich Leukemia Laboratory. A Reiter received Honoraria from Novartis and BMS. BS Bochner is a co‑author on existing and pend‑ ing Siglec‑8‑related patents. If Siglec‑8‑related products are developed in the future, BS Bochner may be entitled to a share of royalties received by Johns Hopkins University on the potential sales of such products. The terms of this arrangement are being managed by Johns Hopkins University in accordance with its conflict of interest policies. This study was sup‑ ported by a research grant of the Medical University of Vienna and by the Division of Intramural Research, NIAID, NIH, Bethesda, MD, USA. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Keywords

  • FIP1L1-PDGFRA
  • classification
  • eosinophilic leukemia
  • hypereosinophilia
  • hypereosinophilic syndromes
  • targeted therapy

ASJC Scopus subject areas

  • Hematology

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