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Angioimmunoblastic T-cell lymphoma and hypereosinophilic syndrome with FIP1L1/PDGFRA fusion gene effectively treated with imatinib: A case report.
https://asahikawa-med.repo.nii.ac.jp/records/5918
https://asahikawa-med.repo.nii.ac.jp/records/5918c080ddfc-8726-4b44-bb29-e8f5d1e08d9b
名前 / ファイル | ライセンス | アクション |
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7033.pdf (572.4 kB)
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Item type | 学術雑誌論文 / Journal Article_02(1) | |||||
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公開日 | 2018-07-26 | |||||
タイトル | ||||||
タイトル | Angioimmunoblastic T-cell lymphoma and hypereosinophilic syndrome with FIP1L1/PDGFRA fusion gene effectively treated with imatinib: A case report. | |||||
言語 | ||||||
言語 | eng | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | angioimmunoblastic T-cell lymphoma | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | FIP1L1/PDGFRA | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | hypereosinophilic syndrome | |||||
キーワード | ||||||
主題Scheme | Other | |||||
主題 | imatinib | |||||
資源タイプ | ||||||
資源タイプ識別子 | http://purl.org/coar/resource_type/c_6501 | |||||
資源タイプ | journal article | |||||
著者 |
山本, 昌代
× 山本, 昌代× 生田, 克哉× 土岐, 康通× 畑山, 真弓× 進藤, 基博× 鳥本, 悦宏× 奥村, 利勝 |
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著者 ローマ字 | ||||||
値 | Yamamoto, Masayo | |||||
著者 ローマ字 | ||||||
値 | Ikuta, Katsuya | |||||
著者 ローマ字 | ||||||
値 | Toki, Yasumichi | |||||
著者 ローマ字 | ||||||
値 | Hatayama, Mayumi | |||||
著者 ローマ字 | ||||||
値 | Shindo, Motohiro | |||||
著者 ローマ字 | ||||||
値 | Torimoto, Yoshihiro | |||||
著者 ローマ字 | ||||||
値 | Okumura, Toshikatsu | |||||
書誌情報 |
Medicine 巻 96, 号 36, p. e8001, 発行日 2017-08-01 |
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ISSN | ||||||
収録物識別子タイプ | ISSN | |||||
収録物識別子 | 0025-7974 | |||||
DOI | ||||||
関連タイプ | isIdenticalTo | |||||
識別子タイプ | DOI | |||||
関連識別子 | 10.1097/MD.0000000000008001. | |||||
識別番号 その他 | ||||||
内容記述タイプ | Other | |||||
内容記述 | PMID:28885361 | |||||
抄録 | ||||||
内容記述タイプ | Abstract | |||||
内容記述 | RATIONALE: \nHypereosinophilic syndrome (HES) is a rare disorder characterized by hypereosinophilia and organ damage. Some cases of HES are caused by the FIP1L1/PDGFRA fusion gene and respond to imatinib. FIP1L1/PDGFRA-positive HES occasionally evolves into chronic eosinophilic leukemia or into another form of myeloproliferative neoplasm; however, the development of a malignant lymphoma is very rare. We present a rare case of angioimmunoblastic T-cell lymphoma (AITL) and HES with the FIP1L1/PDGFRA gene rearrangement. PATIENT CONCERNS: \nA man in his 30s presented to our hospital with fever, hypereosinophilia, widespread lymphadenopathy, and splenomegaly. Laboratory tests showed hypereosinophilia, increased soluble interleukin-2 receptor, and increased vitamin B12. Positron-emission tomography with F fluorodeoxyglucose (FDG) showed positive FDG uptake in multiple enlarged lymph nodes throughout the body and the red bone marrow. A bone-marrow biopsy showed hypereosinophilia without dysplasia and an increased number of blasts. The FIP1L1/PDGFRA fusion gene was positive upon fluorescence in situ hybridization (FISH) analysis of the peripheral blood. Furthermore, biopsy of a lymph node from the neck revealed restiform hyperplasia of capillary vessels, with small lymphoma cells arranged around the capillaries. Lymphoma cells were positive for CD3, CD4, and CD10, and negative for CD20. Lymphoma cells were also positive for the FIP1L1/PDGFRA fusion gene by FISH analysis. DIAGNOSES: \nFrom these findings, the patient was diagnosed with HES and AITL with FIP1L1/PDGFRA. INTERVENTIONS: \nAfter the diagnosis, corticosteroid was administered but was ineffective. Imatinib was then administered. OUTCOMES: \nImatinib was very effective for treating HES and AITL, and complete remission was achieved in both. LESSONS: \nThis report presents the first case in which the FIP1L1/PDGFRA fusion gene was positive both in peripheral blood and lymph nodes, implying the possibility that the tumor cells acquired the FIP1L1/PDGFRA fusion gene in the early stage of hematopoietic progenitor cell developments. Imatinib was very effective in treating both HES and lymphoma, suggesting that the FIP1L1/PDGFRA fusion gene plays a key role in the pathogenesis of both HES and lymphoma. |
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注記 | ||||||
内容記述タイプ | Other | |||||
内容記述 | This is an open access article distributed under the terms of the Creative Commons Attribution-Noncommercial License 4.0 (CCBY-NC), | |||||
資源タイプ | ||||||
内容記述タイプ | Other | |||||
内容記述 | text | |||||
著者版フラグ | ||||||
出版タイプ | VoR | |||||
出版タイプResource | http://purl.org/coar/version/c_970fb48d4fbd8a85 | |||||
フォーマット | ||||||
内容記述タイプ | Other | |||||
内容記述 | application/pdf | |||||
ID(XooNIps) | ||||||
値 | 28885361 | |||||
閲覧数(XooNIps) | ||||||
値 | 353 | |||||
ダウンロード数(XooNIps) | ||||||
値 | 212 |