Abstract |
We previously studied clinico-pathologic features of 89 consecutive adult patients with moderate-to-severe eosinophilia, and reported a FIP1L1-PDGFRA prevalence of 12%. In that series, all 11 FIP1L1-PDGFRA+ patients receiving imatinib achieved a complete response. We now extend our observations through a study of 741 unselected patients with eosinophilia for FIP1L1-PDGFRA, and present longer term follow up data for the imatinib-treated cohort. We also include data for three previously unreported FIP1L1-PDGFRA+ patients. Among the 741 requests, only 21 (3%) were found to carry the FIP1L1-PDGFRA mutation. While all 14 FIP1L1-PDGFRA+ patients receiving imatinib achieved a complete response, the 4 patients who attempted to discontinue imatinib all relapsed. We also find that it is possible to maintain patients in clinical remission with an empirically derived schedule of low-dose (50-100 mg), intermittent (once daily to once weekly) imatinib. Lastly, we present a comprehensive review of the literature pertaining to FIP1L1-PDGFRA in order to address several key aspects of this mutation from a clinical standpoint.
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Authors | A Pardanani, R P Ketterling, C-Y Li, M M Patnaik, A P Wolanskyj, M A Elliott, J K Camoriano, J H Butterfield, G W Dewald, A Tefferi |
Journal | Leukemia research
(Leuk Res)
Vol. 30
Issue 8
Pg. 965-70
(Aug 2006)
ISSN: 0145-2126 [Print] England |
PMID | 16406016
(Publication Type: Journal Article)
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Chemical References |
- Benzamides
- Oncogene Proteins, Fusion
- Piperazines
- Pyrimidines
- mRNA Cleavage and Polyadenylation Factors
- Imatinib Mesylate
- FIP1L1-PDGFRA fusion protein, human
- Receptor, Platelet-Derived Growth Factor alpha
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Topics |
- Adult
- Aged
- Benzamides
- Cohort Studies
- Dose-Response Relationship, Drug
- Drug Administration Schedule
- Eosinophilia
(drug therapy, epidemiology, genetics)
- Follow-Up Studies
- Humans
- Imatinib Mesylate
- Male
- Maximum Tolerated Dose
- Middle Aged
- Mutation
- Oncogene Proteins, Fusion
(genetics)
- Piperazines
(administration & dosage)
- Prevalence
- Pyrimidines
(administration & dosage)
- Receptor, Platelet-Derived Growth Factor alpha
(genetics)
- Recurrence
- Remission Induction
- Treatment Outcome
- mRNA Cleavage and Polyadenylation Factors
(genetics)
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