The DOI matches its Crossref record; 10 of the 10 references it cites were found in a registry or an index.
FIP1L1-PDGFRA fusion: prevalence and clinicopathologic correlates in 89 consecutive patients with moderate to severe eosinophilia
Open accessAbstract
A novel oncogenic mutation (FIP1L1-PDGFRA), which results in a constitutively activated platelet-derived growth factor receptor-α (PDGFRA), has been invariably associated with a primary eosinophilic disorder. The current study examines both the prevalence and the associated clinicopathologic features of this mutation in a cohort of 89 adult patients presenting with an absolute eosinophil count (AEC) of higher than 1.5 × 109/L. A fluorescence in situ hybridization (FISH)–based strategy was used to detect FIP1L1-PDGFRA in bone marrow cells. None of 8 patients with reactive eosinophilia displayed the abnormality, whereas the incidence of FIP1L1-PDGFRA in the remaining 81 patients with primary eosinophilia was 14% (11 patients). None (0%) of 57 patients with the hypereosinophilic syndrome (HES) but 10 (56%) of 19 patients with systemic mast cell disease associated with eosinophilia (SMCD-eos) carried the specific mutation. The bone marrow mast cell infiltration pattern in FIP1L1-PDGFRA+ SMCD-eos was distinctly diffuse with loose tumoral aggregates. Treatment with low-dose imatinib (100 mg/d) produced complete and durable responses in all 8 FIP1L1-PDGFRA+ cases treated. In contrast, only 40% partial response rate was seen in 10 HES cases. FIP1L1-PDGFRA is a relatively infrequent but treatment-relevant mutation in primary eosinophilia that is indicative of an underlying systemic mastocytosis.
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Self-citationApperley, J. F., Gardembas, M., Melo, J. V., Russell-Jones, R., Bain, B. J., Baxter, E. J., Chase, A., Chessells, J. M., Colombat, M., Dearden, C. E., et al. (2002) · New England Journal of Medicine · doi:10.1056/nejmoa020150 · Cited by 630
Pardanani, A., Ketterling, R. P., Brockman, S. R., Flynn, H. C., Paternoster, S. F., Shearer, B. M., Reeder, T. L., Li, C.-Y., Cross, N. C. P., Cools, J., et al. (2003) · Blood · doi:10.1182/blood-2003-05-1627 · Cited by 393
Self-citationKlion, A. D., Noel, P., Akin, C., Law, M. A., Gilliland, D. G., Cools, J., Metcalfe, D. D., & Nutman, T. B. (2003) · Blood · doi:10.1182/blood-2003-01-0006 · Cited by 386
Treatment of hypereosinophilic syndrome with imatinib mesilate
Gleich, G. J., Leiferman, K. M., Pardanani, A., Tefferi, A., & Butterfield, J. H. (2002) · The Lancet · doi:10.1016/s0140-6736(02)08505-7 · Cited by 306
Self-citationImatinib for systemic mast-cell disease
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Self-citationImatinib therapy for hypereosinophilic syndrome and other eosinophilic disorders
Pardanani, A., Reeder, T., Porrata, L. F., Li, C.-Y., Tazelaar, H. D., Baxter, E. J., Witzig, T. E., Cross, N. C. P., & Tefferi, A. (2003) · Blood · doi:10.1182/blood-2002-10-3103 · Cited by 227
Self-citationThe eosinophilias, including the idiopathic hypereosinophilic syndrome
Brito‐Babapulle, F. (2003) · British Journal of Haematology · doi:10.1046/j.1365-2141.2003.04195.x · Cited by 208
Efficacy of imatinib mesylate in the treatment of idiopathic hypereosinophilic syndrome
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Cited by
Arber, D. A., Orazi, A., Hasserjian, R. P., Borowitz, M. J., Calvo, K. R., Kvasnicka, H.-M., Wang, S. A., Bagg, A., Barbui, T., Branford, S., et al. (2022) · Blood · doi:10.1182/blood.2022015850 · Cited by 2,945
Self-citationThe Myeloproliferative Disorders
Campbell, P. J., & Green, A. R. (2006) · New England Journal of Medicine · doi:10.1056/nejmra063728 · Cited by 915
Valent, P., Akin, C., Escribano, L., Födinger, M., Hartmann, K., Brockow, K., Castells, M., Sperr, W. R., Kluin‐Nelemans, H. C., Hamdy, N. A. T., et al. (2007) · European Journal of Clinical Investigation · doi:10.1111/j.1365-2362.2007.01807.x · Cited by 750
Zhang, L. Y., Smith, M. L., Schultheis, B., Fitzgibbon, J., Lister, T. A., Melo, J. V., Cross, N. C. P., & Cavenagh, J. D. (2006) · Leukemia Research · doi:10.1016/j.leukres.2005.08.015 · Cited by 129
Systemic Mastocytosis, Version 2.2019, NCCN Clinical Practice Guidelines in Oncology
Gotlib, J., Gerds, A. T., Bose, P., Castells, M. C., Deininger, M. W., Gojo, I., Gundabolu, K., Hobbs, G., Jamieson, C., McMahon, B., et al. (2018) · Journal of the National Comprehensive Cancer Network · doi:10.6004/jnccn.2018.0088 · Cited by 106
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Metadata
- Authors
- Pardanani, A., Brockman, S. R., Paternoster, S. F., Flynn, H. C., Ketterling, R. P., Lasho, T. L., Ho, C.-L., Li, C.-Y., Dewald, G. W., & Tefferi, A.
- Venue
- Blood
- Publisher
- American Society of Hematology
- Published
- 2004-11-15
- Volume
- 104
- Issue
- 10
- Pages
- 3038-3045
- ISSN
- 0006-4971 (ISSN-L)
- 1528-0020
- OpenAlex
- W2078398386
- Cited by
- 309
- References
- 10
- FWCI
- 18.21
- Type
- journal article
- Language
- en
- Open access
- bronze
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- CoChat. (2026). Citation check: FIP1L1-PDGFRA fusion: prevalence and clinicopathologic correlates in 89 consecutive patients with moderate to severe eosinophilia. https://app.cochat.ai/papers/10.1182/blood-2004-03-0787
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- Pardanani, A., Brockman, S. R., Paternoster, S. F., Flynn, H. C., Ketterling, R. P., Lasho, T. L., Ho, C.-L., Li, C.-Y., Dewald, G. W., & Tefferi, A. (2004). FIP1L1-PDGFRA fusion: prevalence and clinicopathologic correlates in 89 consecutive patients with moderate to severe eosinophilia. Blood. https://doi.org/10.1182/blood-2004-03-0787
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
