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著作: [賀川 久美子]/[中村 信元]/八木 ひかる/[曽我部 公子]/髙橋 真美子/丸橋 朋子/宇髙 憲吾/[藤井 志朗]/[三木 浩和]/[安倍 正博]/同系骨髄移植が著効した最重症再生不良性貧血の1例/[四国医学雑誌]

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EID
303542
EOID
1043622
Map
0
LastModified
2022年5月9日(月) 14:02:34
Operator
三好 小文
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TRUE
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Owner
中村 信元
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種別 必須 学術論文(審査論文)
言語 必須 日本語
招待 推奨
審査 推奨
カテゴリ 推奨 研究
共著種別 推奨
学究種別 推奨
組織 推奨
著者 必須
  1. 賀川 久美子
    役割 任意
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  2. 中村 信元([徳島大学.大学院医歯薬学研究部.医学域.連携研究部門(医学域).寄附講座系(医学域).実践地域診療・医科学])
    役割 任意
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    学籍番号 推奨
  3. (日) 八木 ひかる
    役割 任意
    貢献度 任意
    学籍番号 推奨
  4. 曽我部 公子([徳島大学.病院.先端医科医療開発研究プロジェクト]/[徳島大学.病院.診療科.内科.血液内科]/[徳島大学.大学院医歯薬学研究部.医学域.医科学部門.内科系.血液・内分泌代謝内科学])
    役割 任意
    貢献度 任意
    学籍番号 推奨
  5. (日) 髙橋 真美子
    役割 任意
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    学籍番号 推奨
  6. (日) 丸橋 朋子
    役割 任意
    貢献度 任意
    学籍番号 推奨
  7. (日) 宇髙 憲吾
    役割 任意
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    学籍番号 推奨
  8. 藤井 志朗([徳島大学.病院.先端医科医療開発研究プロジェクト]/[徳島大学.病院.診療科.内科.血液内科])
    役割 任意
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    学籍番号 推奨
  9. 三木 浩和([徳島大学.病院.中央診療施設等.輸血・細胞治療部])
    役割 任意
    貢献度 任意
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  10. 安倍 正博([徳島大学.大学院医歯薬学研究部.医学域.医科学部門.内科系.血液・内分泌代謝内科学])
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題名 必須

(英) Successful treatment of refractory severe aplastic anemia with bone marrow transplantation from a genetically identical twin

(日) 同系骨髄移植が著効した最重症再生不良性貧血の1例

副題 任意
要約 任意

(英) Aplastic anemia is a bone marrow failure caused by severely curtailed hematopoietic stem cells (HSCs) and dysregulation of ambient immune cells. Immumo-suppressive conditioning followed by allogeneic HSC transplantation is currently a mainstay in treatment for patients at a younger age or those refractory to conventional immunosuppressive remedies. Syngeneic HSC transplantation appears to be promising, but has been very rarely performed ; therefore, its impact on a long-term outcome as well as the best preparative measures for HSC engraftment and immune amelioration are still largely unknown. Here, we reported a successful and beneficial syngeneic HSC transplantation for a refractory case with very severe aplastic anemia. A 30-year-old female presented high fever after tooth extraction, and was diagnosed with very severe aplastic anemia. Cyclosporine and anti-thymocyte globulin were initiated, but showed no hematological effects. After obtaining an informed consent, she underwent bone marrow transplantation from a genetically identical twin following an non-myeloablative conditioning regimen consisted of cyclophosphamide (750mg/m2, 4 days), fludarabine phosphate (25mg/m2, 4 days). Cyclosporine was given for acute GvHD prophylaxis. Her neutrophils recovered over 500/μl on 12 days after the transplantation, and her blood counts have been maintained in a normal range over 7 years thereafter. Although a fate of HSCs from a genetically identical twin and an immune response of ambient cells in the bone marrow in recipients remains largely unknown, from the present case and previously reported cases, we dare to recommend immunoablative conditioning and acute GvHD prophylaxis in syngeneic HSC transplantation for a refractory case with aplastic anemia for better engraftment and sustained onward recovery of hematopoiesis.

(日) Aplastic anemia is a bone marrow failure caused by severely curtailed hematopoietic stem cells (HSCs) and dysregulation of ambient immune cells. Immumo-suppressive conditioning followed by allogeneic HSC transplantation is currently a mainstay in treatment for patients at a younger age or those refractory to conventional immunosuppressive remedies. Syngeneic HSC transplantation appears to be promising, but has been very rarely performed ; therefore, its impact on a long-term outcome as well as the best preparative measures for HSC engraftment and immune amelioration are still largely unknown. Here, we reported a successful and beneficial syngeneic HSC transplantation for a refractory case with very severe aplastic anemia. A 30-year-old female presented high fever after tooth extraction, and was diagnosed with very severe aplastic anemia. Cyclosporine and anti-thymocyte globulin were initiated, but showed no hematological effects. After obtaining an informed consent, she underwent bone marrow transplantation from a genetically identical twin following an non-myeloablative conditioning regimen consisted of cyclophosphamide (750mg/m2, 4 days), fludarabine phosphate (25mg/m2, 4 days). Cyclosporine was given for acute GvHD prophylaxis. Her neutrophils recovered over 500/μl on 12 days after the transplantation, and her blood counts have been maintained in a normal range over 7 years thereafter. Although a fate of HSCs from a genetically identical twin and an immune response of ambient cells in the bone marrow in recipients remains largely unknown, from the present case and previously reported cases, we dare to recommend immunoablative conditioning and acute GvHD prophylaxis in syngeneic HSC transplantation for a refractory case with aplastic anemia for better engraftment and sustained onward recovery of hematopoiesis.

キーワード 推奨
  1. (英) aplastic anemia
  2. (英) genetically identical twin
  3. (英) syngeneic hematopoietic stem cell transplantation
発行所 推奨 徳島医学会
誌名 必須 四国医学雑誌([徳島医学会])
(pISSN: 0037-3699)
ISSN 任意 0037-3699
ISSN: 0037-3699 (pISSN: 0037-3699)
Title: 四国医学雑誌
Supplier: 徳島大学
Publisher: Tokushima Igakkai
 (Webcat Plus  (医中誌Web  (Scopus (Scopus information is found. [need login])
必須 70
必須 3,4
必須 77 80
都市 任意
年月日 必須 2014年 6月 初日
URL 任意 https://repo.lib.tokushima-u.ac.jp/109757
DOI 任意
PMID 任意
CRID 任意 1050001337464993408
NAID 120005820008
WOS 任意
Scopus 任意
機関リポジトリ 109757
評価値 任意
被引用数 任意
指導教員 推奨
備考 任意