1. PDGFRalpha/FIP1L1-positive chronic eosinophilic leukemia presenting with retro-orbital localization: efficacy of imatinib treatment
- Author
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Fortunato Morabito, Emanuela Ottaviani, Eugenio Lucia, Carlo Gentile, Michela Rondoni, Maria Grazia Bisconte, Giovanni Martinelli, Ernesto Vigna, Carla Mazzone, Antonio Armentano, Massimo Gentile, Vigna E, Lucia E, Gentile M, Mazzone C, Bisconte MG, Gentile C, Armentano A, Ottaviani E, Rondoni M, Martinelli G, and Morabito F.
- Subjects
Male ,Cancer Research ,medicine.medical_specialty ,Receptor, Platelet-Derived Growth Factor alpha ,Oncogene Proteins, Fusion ,medicine.drug_class ,Antineoplastic Agents ,Toxicology ,IMATINIB ,Gastroenterology ,Tyrosine-kinase inhibitor ,Piperazines ,Internal medicine ,Hypereosinophilic Syndrome ,medicine ,Humans ,Pharmacology (medical) ,Pharmacology ,mRNA Cleavage and Polyadenylation Factors ,Chronic eosinophilic leukemia ,ABL ,Hypereosinophilic syndrome ,business.industry ,Reverse Transcriptase Polymerase Chain Reaction ,Imatinib ,Hypoesthesia ,Middle Aged ,medicine.disease ,Magnetic Resonance Imaging ,Imatinib mesylate ,Pyrimidines ,Oncology ,CHRONIC EOSINOPHILIC LEUKEMIA ,Benzamides ,Cancer research ,Imatinib Mesylate ,Orbital Neoplasms ,medicine.symptom ,business ,Tomography, X-Ray Computed ,Tyrosine kinase ,medicine.drug - Abstract
The fusion protein between the platelet-derived growth factor receptor alpha (PDGFRalpha, P) gene and the Fip1-like1 (FIP1L1, F) may be identified in 14 to 60% of HES and it indicates a clonal hypereosinophilic syndrome called F/P-positive CEL. We herein report a case of F/P-positive CEL with retro-orbital localization, who was successfully treated with imatinib. CASE REPORT: A 53-year-old male presented an absolute eosinophil count of 25,000/mm(3), anemia (Hb 10.2 g/dl) and a moderate increase in the platelet count (571,000/mm(3)). A clinical examination revealed left exophthalm, associated with diffuse hypoesthesia and diplopia. A CT scan of orbits showed a lesion located in the lachrymal fossa of the left orbit with intra- and extra-conical extension. Molecular analysis excluded the presence of bcr/abl transcript while a F/P fusion tyrosine kinase signal was documented. Imatinib mesylate (IM) was started and, after 7 days of treatment eosinophil count significantly declined along with a dramatic reduction of the left exophthalm. IM dosage was increased up to 300 mg/day. The drug was well tolerated with an initial modest haematological toxicity. The left exophthalm, as well as hypoesthesia and diplopia, disappeared after IM therapy. MRI showed a clear reduction of the intra- and extra-conical growth process. BM molecular signal of the F/P fusion gene resulted undetectable after 4 weeks of treatment. CONCLUSION: In our case, the diagnosis of FIPIL1-PDGFRA-positive CEL and IM therapy has allowed the patient to experience an excellent clinical therapeutic result, avoiding surgical treatment of the retro-orbital mass.
- Published
- 2007