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• W2589122028 abstract "FIP1L1/PDGFRa fusion gene is generated by a microdeletion on chromosome 4q12, and associates with developing hypereosinophilic syndrome (HES) and chronic eosinophilic leukemia (CEL). Some patients of acute myeloid leukemia and lymphoblastic leukemia have this gene, but it is very rare in patients of malignant lymphoma. Now, we report the patients of malignant lymphoma and HES with FIP1L1/PDGFRa fusion gene.Case: A 30's-year-old man visited our hospital because of fever and lymphadenopathy. His blood test showed leukocytosis (WBC 12890/µl) with hypereosinophilia (Eos 55.0%), and FIP1L1/PDGFRα fusion gene by FISH analysis. His bone marrow showed hypereosinophilia without dysplasia and increasing blasts, and T-cell receptor (TCR) gene rearrangement was negative. Chromosomal analysis revealed a normal karyotype in G-band. Positron emission tomography with 18F-fluorodeoxyglucose (FDG-PET) revealed multiple lymphadenopathy in whole body and red bone marrow with uptake of FDG. We then performed lymph node biopsy of the neck and diagnosed angioimmunoblastic T-cell lymphoma (AITL). TCR gene rearrangement and FIP1L1/PDGFRa fusion gene were positive by western blotting and by FISH analysis in lymph node, respectively. From these results, we diagnosed AITL and HES with FIP1L1/PDGFRa. Corticosteroid (0.5 mg/kg/day) was administered but was not effective. So we administered imatinib (100 mg/day), and his symptoms, hypereosinophilia and lymphadenopathy were improved quickly. Clinical course was very good by only imatinib, and bone marrow asipiration and FDG-PET after three months revealed complete remission of both HES and AITL.Imatinib is reported to be very effective for HES and CEL patients with FIP1L1/PDGFRa fusion gene. This case showed that imatinib was very effective for not only HES but also AITL with FIP1L1/PDGFRa fusion gene. FIP1L1/PDGFRa fusion gene is generated by a microdeletion on chromosome 4q12, and associates with developing hypereosinophilic syndrome (HES) and chronic eosinophilic leukemia (CEL). Some patients of acute myeloid leukemia and lymphoblastic leukemia have this gene, but it is very rare in patients of malignant lymphoma. Now, we report the patients of malignant lymphoma and HES with FIP1L1/PDGFRa fusion gene. Case: A 30's-year-old man visited our hospital because of fever and lymphadenopathy. His blood test showed leukocytosis (WBC 12890/µl) with hypereosinophilia (Eos 55.0%), and FIP1L1/PDGFRα fusion gene by FISH analysis. His bone marrow showed hypereosinophilia without dysplasia and increasing blasts, and T-cell receptor (TCR) gene rearrangement was negative. Chromosomal analysis revealed a normal karyotype in G-band. Positron emission tomography with 18F-fluorodeoxyglucose (FDG-PET) revealed multiple lymphadenopathy in whole body and red bone marrow with uptake of FDG. We then performed lymph node biopsy of the neck and diagnosed angioimmunoblastic T-cell lymphoma (AITL). TCR gene rearrangement and FIP1L1/PDGFRa fusion gene were positive by western blotting and by FISH analysis in lymph node, respectively. From these results, we diagnosed AITL and HES with FIP1L1/PDGFRa. Corticosteroid (0.5 mg/kg/day) was administered but was not effective. So we administered imatinib (100 mg/day), and his symptoms, hypereosinophilia and lymphadenopathy were improved quickly. Clinical course was very good by only imatinib, and bone marrow asipiration and FDG-PET after three months revealed complete remission of both HES and AITL. Imatinib is reported to be very effective for HES and CEL patients with FIP1L1/PDGFRa fusion gene. This case showed that imatinib was very effective for not only HES but also AITL with FIP1L1/PDGFRa fusion gene." @default.
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• W2589122028 date "2016-11-01" @default.
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• W2589122028 title "AITL and HES with FIP1L1/PDGFRa fusion gene effectively treated with imatinib" @default.
• W2589122028 doi "https://doi.org/10.1093/annonc/mdw524.014" @default.
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