Primary eosinophilic disorders

A concise review

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

Primary eosinophilic disorders include hypereosinophilic syndrome (HES); chronic eosinophilic leukemia, not otherwise categorized (CEL-NOC); platelet-derived growth factor receptor (PDGFR)-rearranged myeloid neoplasms; and other myeloid malignancies associated with prominent blood eosinophilia. According to the World Health Organization consensus criteria, the diagnosis of HES requires the absence of clonal cytogenetic or molecular markers of an underlying myeloid or lymphoid neoplasm. CEL-NOC constitutes an HES-like phenotype associated with an abnormal karyotype or excess blasts in blood (> 2%) or bone marrow (> 5%). HES and CEL-NOC are considered distinct from molecularly defined eosinophilic disorders, such as those associated with activating mutations of PDGFR (PDGFRA and PDGFRB) and fibroblast growth factor receptor-1. This is an important distinction because PDGFR-mutated but not other eosinophilic neoplasms are effectively treated with imatinib. Current management in HES includes observation only for asymptomatic patients with no evidence of organ damage, systemic corticosteroid therapy for acute control of symptoms, and interferon-alfa-2a or hydroxyurea as steroid-sparing agents. In patients with HES who are refractory to usual therapy and have life-threatening disease complications, the use of investigational drugs such as alemtuzumab or mepolizumab might be considered, but data on long-term efficacy and safety are limited.

Original languageEnglish (US)
Pages (from-to)37-43
Number of pages7
JournalCurrent Hematologic Malignancy Reports
Volume3
Issue number1
DOIs
StatePublished - Mar 2008

Fingerprint

Hypereosinophilic Syndrome
Platelet-Derived Growth Factor Receptors
Neoplasms
Receptor, Fibroblast Growth Factor, Type 1
Investigational Drugs
Platelet-Derived Growth Factor beta Receptor
Abnormal Karyotype
Hydroxyurea
Eosinophilia
Cytogenetics
Adrenal Cortex Hormones
Bone Marrow
Steroids
Observation
Phenotype
Safety
Mutation
Therapeutics
Pdgfra-Associated Chronic Eosinophilic Leukemia

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research

Cite this

Primary eosinophilic disorders : A concise review. / Pardanani, Animesh D; Tefferi, Ayalew.

In: Current Hematologic Malignancy Reports, Vol. 3, No. 1, 03.2008, p. 37-43.

Research output: Contribution to journalArticle

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