徳島大学 教育・研究者情報データベース(EDB)

1. 賀川 久美子

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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])

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3. (日) 八木 ひかる

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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])

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5. (日) 髙橋 真美子

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6. (日) 丸橋 朋子

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7. (日) 宇髙 憲吾

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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])

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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])

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10. 安倍 正博

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(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

(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.

1. (英) aplastic anemia
2. (英) genetically identical twin
3. (英) syngeneic hematopoietic stem cell transplantation