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Contemporary consensus proposal on criteria and classification of eosinophilic disorders and related syndromes

Published:March 29, 2012DOI:https://doi.org/10.1016/j.jaci.2012.02.019
      Eosinophilia is an important indicator of various neoplastic and nonneoplastic conditions. Depending on the underlying disease and mechanisms, eosinophil infiltration can lead to organ dysfunction, clinical symptoms, or both. During the past 2 decades, several different classifications of eosinophilic disorders and related syndromes have been proposed in various fields of medicine. Although criteria and definitions are, in part, overlapping, no global consensus has been presented to date. The Year 2011 Working Conference on Eosinophil Disorders and Syndromes was organized to update and refine the criteria and definitions for eosinophilic disorders and to merge prior classifications in a contemporary multidisciplinary schema. A panel of experts from the fields of immunology, allergy, hematology, and pathology contributed to this project. The expert group agreed on unifying terminologies and criteria and a classification that delineates various forms of hypereosinophilia, including primary and secondary variants based on specific hematologic and immunologic conditions, and various forms of the hypereosinophilic syndrome. For patients in whom no underlying disease or hypereosinophilic syndrome is found, the term hypereosinophilia of undetermined significance is introduced. The proposed novel criteria, definitions, and terminologies should assist in daily practice, as well as in the preparation and conduct of clinical trials.

      Key words

      Abbreviations used:

      BM (Bone marrow), CEL (Chronic eosinophilic leukemia), FGFR (Fibroblast growth factor receptor), HE (Hypereosinophilia), HEN (Primary (clonal/neoplastic) hypereosinophilia produced by apparently clonal (neoplastic) eosinophils), HER (Secondary (reactive) hypereosinophilia), HES (Hypereosinophilic syndrome), HEUS (Hypereosinophilia of undetermined significance), ICOG-EO (International Cooperative Working Group on Eosinophil Disorders), MDS (Myelodysplastic syndrome), MPN (Myeloproliferative neoplasm), PDGFR (Platelet-derived growth factor receptor), PDGFRA (Platelet-derived growth factor receptor α), PDGFRB (Platelet-derived growth factor receptor β), SM (Systemic mastocytosis), WHO (World Health Organization)
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