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Imatinib mesylate as a novel treatment option for hypereosinophilic syndrome: two case reports and a comprehensive review of the literature
- Source :
- Annals of hematology. 85(1)
- Publication Year :
- 2005
-
Abstract
- Hypereosinophilic syndromes (HES) are a heterogenous group of rare disorders characterized by sustained and otherwise unexplained overproduction of eosinophils with organ involvement and consecutive dysfunction. Recent reports document the efficacy of imatinib mesylate in a large proportion of HES patients (65%). Rearrangements involving the platelet-derived growth factor receptor genes (PDGFRA and PDGFRB), both tyrosine kinase receptors, have been demonstrated to be pathogenetically linked to the dysregulated clonal overproduction of eosinophils. This refined hypothesis has been confirmed by the discovery of the novel FIP1L1-PDGFRA fusion gene, which is a gain-of-function gene on chromosome 4q12. Its product is an imatinib-sensitive tyrosine kinase, which can be found in a subset of patients with HES, particularly in those responding to treatment with imatinib mesylate. Here, we sum up recent knowledge of clinical features, pathophysiology and novel treatment aspects of HES by performing a comprehensive search of the available literature and report on 94 patients. We particularly address the issue of organ involvement and specific characteristics of the variable clinical pictures. In addition, two cases will be presented, which illustrate typical clinical scenarios and treatment outcome.
- Subjects :
- Oncology
Adult
Male
medicine.medical_specialty
Receptor, Platelet-Derived Growth Factor alpha
Oncogene Proteins, Fusion
PDGFRB
PDGFRA
Receptor tyrosine kinase
Piperazines
Receptor, Platelet-Derived Growth Factor beta
Internal medicine
Hypereosinophilic Syndrome
Medicine
Humans
Protein Kinase Inhibitors
Aged
mRNA Cleavage and Polyadenylation Factors
Chronic eosinophilic leukemia
Hematology
biology
business.industry
Hypereosinophilic syndrome
Remission Induction
General Medicine
medicine.disease
Imatinib mesylate
Pyrimidines
Immunology
Benzamides
biology.protein
Imatinib Mesylate
Female
Chromosomes, Human, Pair 4
business
Tyrosine kinase
Subjects
Details
- ISSN :
- 09395555
- Volume :
- 85
- Issue :
- 1
- Database :
- OpenAIRE
- Journal :
- Annals of hematology
- Accession number :
- edsair.doi.dedup.....2a92e56ab7315543de3448f4a576a6c9