Skip Navigation LinksHome > March 2013 - Volume 20 - Issue 2 > Eosinophilic myeloid neoplasms
Current Opinion in Hematology:
doi: 10.1097/MOH.0b013e32835d81bf
MYELOID DISEASE: Edited by Martin S. Tallman

Eosinophilic myeloid neoplasms

Noel, Pierre; Mesa, Ruben A.

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Abstract

Purpose of review: In 2012, idiopathic hypereosinophilic syndrome (HES) is still the prevalent diagnosis in patients with persistent eosinophilia, in which a primary or secondary cause of eosinophilia has not been identified. HES is considered a provisional diagnosis until a primary or secondary cause of hypereosinophilia is established. The discovery of imatinib-sensitive fusion proteins in a subset of patients with hypereosinophilia has changed the way we approach the diagnosis and treatment of eosinophilic myeloid neoplasms [eosinophilic myeloproliferative neoplasms (MPNs)]. Despite the recent diagnostic developments, diagnosis of hypereosinophilic MPN is only made in 10–20% of patients with persistent primary hypereosinophilia.

Recent findings: In 2008 the World Health Organization (WHO) established a semi-molecular classification of hypereosinophilic MPNs. The discovery of PDGFRA, PDGFRB, FGFR1, JAK-2, and FLT3 fusion proteins in patients with eosinophilic MPNs provide opportunities for targeted therapy. Patients with hypereosinophilic MPNs associated with PDGFRA and PDGFRB fusion genes are responsive to imatinib.

Summary: Ongoing research continues to expand our understanding of the pathophysiology of persistent primary hypereosinophilia and clarify the boundaries between some of these disorders. A key challenge is to identify new targets for therapy and limit the number of patients who are classified as having HES.

© 2013 Lippincott Williams & Wilkins, Inc.

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