ID 109757
Title Transcription
ドウケイ コツズイ イショク ガ チョコウ シタ サイジュウショウ サイセイ フリョウセイ ヒンケツ ノ 1レイ
Title Alternative
Successful treatment of refractory severe aplastic anemia with bone marrow transplantation from a genetically identical twin
Author
Kagawa, Kumiko Department of Medicine and Bioregulatory Sciences, Graduate School of Medical Sciences, University of Tokushima Tokushima University Educator and Researcher Directory KAKEN Search Researchers
Nakamura, Shingen Department of Medicine and Bioregulatory Sciences, Graduate School of Medical Sciences, University of Tokushima Tokushima University Educator and Researcher Directory KAKEN Search Researchers
Yagi, Hikaru Department of Medicine and Bioregulatory Sciences, Graduate School of Medical Sciences, University of Tokushima
Sogabe, Kimiko Department of Medicine and Bioregulatory Sciences, Graduate School of Medical Sciences, University of Tokushima Tokushima University Educator and Researcher Directory
Takahashi, Mamiko Department of Medicine and Bioregulatory Sciences, Graduate School of Medical Sciences, University of Tokushima
Maruhashi, Tomoko Department of Medicine and Bioregulatory Sciences, Graduate School of Medical Sciences, University of Tokushima
Udaka, Kengo Department of Medicine and Bioregulatory Sciences, Graduate School of Medical Sciences, University of Tokushima
Fujii, Shirou Department of Medicine and Bioregulatory Sciences, Graduate School of Medical Sciences, University of Tokushima Tokushima University Educator and Researcher Directory KAKEN Search Researchers
Miki, Hirokazu Department of transfusion and cell therapy, Tokushima University Hospital Tokushima University Educator and Researcher Directory KAKEN Search Researchers
Abe, Masahiro Department of Medicine and Bioregulatory Sciences, Graduate School of Medical Sciences, University of Tokushima Tokushima University Educator and Researcher Directory KAKEN Search Researchers
Keywords
aplastic anemia
genetically identical twin
syngeneic hematopoietic stem cell transplantation
Content Type
Journal Article
Description
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.
Journal Title
四国医学雑誌
ISSN
00373699
NCID
AN00102041
Publisher
徳島医学会
Volume
70
Issue
3-4
Start Page
77
End Page
80
Sort Key
77
Published Date
2014-08-25
EDB ID
FullText File
language
jpn
TextVersion
Publisher
departments
University Hospital
Medical Sciences