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Non-myeloablative allogeneic stem cell transplantation in patients with systemic sclerosis

Research Project

Project/Area Number 16590983
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field 膠原病・アレルギー・感染症内科学
Research InstitutionKYUSHU UNIVERSITY

Principal Investigator

NISHIMURA Junji  Kyushu University, Medical Institute of Bioregulation, Professor, 生体防御医学研究所, 教授 (20112336)

Co-Investigator(Kenkyū-buntansha) SHIOKAWA Satoshi  Kyushu University, Kyushu University Hospital, Assistant Professor, 大学病院, 講師 (20215940)
MOTOMURA Seiichi  Kyushu University, Medical Institute of Bioregulation, Research Associate, 生体防御医学研究所, 助手 (40304828)
Project Period (FY) 2004 – 2005
Project Status Completed (Fiscal Year 2005)
Budget Amount *help
¥3,600,000 (Direct Cost: ¥3,600,000)
Fiscal Year 2005: ¥1,400,000 (Direct Cost: ¥1,400,000)
Fiscal Year 2004: ¥2,200,000 (Direct Cost: ¥2,200,000)
Keywordssystemic sclerosis / hematopoietic stem cell transplantation / alogeneic transplantation / GVHD / 全身性強皮症 / 同種造血幹細胞移植 / 移植片対宿主病
Research Abstract

Systemic sclerosis (SSc) is an autoimmune disease which is characteristic of diffuse scleroderma and visceral organ fibrosis. Various kinds of therapies including immunosuppressive drugs have been attempted yet all proved ineffective. In order to reconstitute the immune system and cure SSc patients, we started a clinical trial of nonmyeloablative hematopoietic stem cell transplantation (NST) for the treatment of SSc. The inclusion criteria consisted of an age younger than 70 years, SSc as defined by Ministry of Health, Labor, and Welfare, a duration of less than 4 years since the diagnosis of SSc, a skin score of more than 15 points, and moderate organ failure. We herein describe the first patient to take part in this trial. A52-year-old female suffered from scleroderma for 3years and we diagnosed to have SSc. Although she was treated with prednisolone, cyclophosphamide, penicilamine, and prostagrandin, the scleroderma was progressive (skin score 30 points) and the interstitial pneumon … More ia also worsened (%VC 66.5%, serum KL-6 level 1080U/mL). In November 2003, we enrolled her as the first patient to undergo NST after her informed consent was obtained. After conditioning therapy consisting of low dose TBI (200 cGy) and fludarabin e (30mg/m^2, 3days), G-CSF-mobilized peripheral blood stem cells from an HLA- identical brother were infused. Graft-versus-host disease (GVHD) prophylaxis consisted of cyclosporine and mycophenolate mofetil. No blood transfusion was performed. Complete donor chimerism was obtained and has continued since 1 month after NST. The cyclosporine dosage was tapered gradually, and no apparent acute or chronic GVHD was seen. Three months after NST, the patient's skin score improved to 19 points and a skin biopsy showed mild lymphocyte infiltration. At 8 months after NST, the area of interstitial pneumonia decreased according to the computed tomography findings and the %VC levels and serum KL-6 level were both found to have substantially improved (70.3% and 746U/mL, respectively). 1-year after NST, she developed chronic GVHD in oral mucosa and membranous nephropathy in kidneys. Although the duration after NST is still short, the clinical course for this patient is presently appears to be promising. Less

Report

(3 results)
  • 2005 Annual Research Report   Final Research Report Summary
  • 2004 Annual Research Report
  • Research Products

    (29 results)

All 2006 2005 2004

All Journal Article (27 results) Book (2 results)

  • [Journal Article] Steroid Myopathy : Evaluation of fiber atropy with T2 relaxatio time-rabbit and human study.2006

    • Author(s)
      Hatakenaka M, Soeda H, Okafuji T, Yabuuchi H, Shiokawa S, Nishimura J, Honda H
    • Journal Title

      Radiology 238(2)

      Pages: 650-657

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Steroid Myopathy : Evaluation of fiber atropy with T2 relaxation time -rabitt and human study.2006

    • Author(s)
      Hatakenaka M, Soeda H, Okafuji T, Yabuuch i H, Shiokawa S, Nishimura J, Honda H
    • Journal Title

      Radiology 238(2)

      Pages: 650-657

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Steroid Myopathy : Evaluation of fiber atropy with T2 relaxation time-rabbit and human study.2006

    • Author(s)
      Hatakenaka M, Soeda H, Okafuji T, Yabuuchi H, Shiokawa S, Nishimura J, Honda H
    • Journal Title

      Radiology 238(2)

      Pages: 650-657

    • Related Report
      2005 Annual Research Report
  • [Journal Article] Detection of unique neutrophil non-muscle myosin heavy chain-A localization by immunofluorescence analysis in MYH9 disorder presented with mactothrombocytopenia without leukocyte inclusions and deafness2005

    • Author(s)
      Kunishima S, Matsushita T, Shiratsuchi M, Ikuta T, Nishimura J, Hamaguchi M, Naoe T, Saito H
    • Journal Title

      Eur J Harmatol 74

      Pages: 1-5

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Selective cyclooxigenase 2 inhibitor NS-398 induces apoptosis in myeloma cells via a Bcl-2 independent pathway2005

    • Author(s)
      Zang M, Abe Y, Matsushima T, Nishimura J, Nawata H, Muta K
    • Journal Title

      Leuk Lymph 46(3)

      Pages: 425-433

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] S Fludarabine-based conditioning used in successful bone marrow transplantation from an unrelated donor in a heavily transfused patient with severe aplastic anemia.2005

    • Author(s)
      Abe Y, Matsushima T, Tachikawa Y, Nagasawa E, Nishimura J, Nawate H, Muta K
    • Journal Title

      Int J Hematol 81(1)

      Pages: 81-82

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] A novel mechanism in suppression of erythropoiesis during inflammation : a crucial role of RCAS12005

    • Author(s)
      Suehiro Y, Muta K, Nakashima M, Abe Y, Shiratsuchi M, Shiokawa S, Ikuyama S, Yoshikawa Y, Watanabe T, Nishimura J
    • Journal Title

      Eur J Harmatol 74

      Pages: 365-373

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Pivotal role of peroxisome proliferators-activated receptor γ(PPARγ) in regulation of erythroid progenitor cell proliferatio and differentiation.2005

    • Author(s)
      Nagasawa E, Abe Y, Nishimura J, Yanase T, Nawata H, Muta K
    • Journal Title

      Exp Hematol 33

      Pages: 857-864

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Molecular remission by nonmyeloablative stem cell transplantatio after autologous peripheral blood stem cell transplantation in a patient with multiple myeloma.2005

    • Author(s)
      Nakashima Y, Shiratsuchi M, Tani K, Abe Y, Muta K, Shiokawa S, Nishimura J
    • Journal Title

      Leuk. Lymphoma 46(8)

      Pages: 1217-1222

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Expression of adipose differentiation-related protein (ADRP) is conjointly regulated by PU.1 and AP-1 in macrophages.2005

    • Author(s)
      Wei P, Taniguchi S, Sakai T, Imamura M, Inokuchi T, Nawata H, Oda S, Nakabeppu Y, Nishimura J, Ikuyama S
    • Journal Title

      J Biochem 138

      Pages: 399-412

    • NAID

      10017347543

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Detection of unique neutrophil non-muscle myosin heavy chain-A localization by immunofluorescence analysis in MYH9 disorder presented with macrothrombocytopenia without leukocyte inclusions and deafness.2005

    • Author(s)
      Kunishima S, Matsushita T, Shiratsuchi M, Ikuta T, Nishimura J, Hamaguchi M, Naoe T, Saito H
    • Journal Title

      Eur J Haematol 74

      Pages: 1-5

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Selective cyclooxigenase 2 inhibitor NS-398 induces apoptosis in myeloma cells via a Bcl-2 independent pathway.2005

    • Author(s)
      Zang M, Abe Y, Matsushima T, Nishimura J, Nawata H, Muta K
    • Journal Title

      Leuk Lymph 46(3)

      Pages: 425-433

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Fludarabine-based conditioning used in successful bone marrow transplantation from an unrelated donor in a heavily transfused patient with severe aplastic anemia.2005

    • Author(s)
      Abe Y, Matsushima T, Tachikawa Y, Nagasawa E, Nishimura J, Nawata H, Muta K
    • Journal Title

      Int J Hematol 81(1)

      Pages: 81-82

    • NAID

      10014282748

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] A novel mechanism in suppression of erythropoiesis during inflammation : a crucial role of RCAS1 Eur J Hematol2005

    • Author(s)
      Suehiro Y, Muta K, Nakashima M, Abe Y, Shiratsuchi M, Shiokawa S, Ikuyama S, Yoshikawa Y, Watanabe T, Nishimura J
    • Journal Title

      Eur J Hematol 74

      Pages: 365-373

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Journal Article] Pivotal role of peroxisome proliferators-activated receptor γ (PPARγ) in regulation of erythroid progenitor cell proliferation and differentiation.2005

    • Author(s)
      Nagasawa E, Abe Y, Nishimura J, Yanase T, Nawata H, Muta K
    • Journal Title

      Exp Hematol 33

      Pages: 857-864

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Annual Research Report 2005 Final Research Report Summary
  • [Journal Article] Molecular remission by nonmyeloablative stem cell transplantation after autologous peripheral blood stem cell transplantation in a patient with multiple myeloma.2005

    • Author(s)
      Nakashima Y, Shiratsuchi M, Tani K, Abe Y, Muta K, Shiokawa S, Nishimura J
    • Journal Title

      Leuk.Lymphoma 46(8)

      Pages: 1217-1222

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Annual Research Report 2005 Final Research Report Summary
  • [Journal Article] Expression of adipose differentiation-related protein (ADRP) is conjointly regulated by PU.1 and AP-1 in macrophages.2005

    • Author(s)
      Wei P, Taniguchi S, Sakai Y, Imamura M, Inokuchi T, Nawata H, Oda S, Nakabeppu Y, Nishimura J, Ikuyama S
    • Journal Title

      J Biochem 138

      Pages: 399-412

    • NAID

      10017347543

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2005 Annual Research Report 2005 Final Research Report Summary
  • [Journal Article] Detection of unique neutrophil non-muscle myosin heavy chain-A localization by immunofluorescence analysis in MYH9 disorder presented with macrothrombocytopenia without leukocyte inclusions and deafness2005

    • Author(s)
      Kunishima S, Matsushita T, Shiratsuchi M, Ikuta T, Nishimura J, Hamaguchi M, Naoe T, Saito H
    • Journal Title

      Eur J Haematol 74

      Pages: 1-5

    • Related Report
      2005 Annual Research Report
  • [Journal Article] Selective cyclooxigenase 2 inhibitor NS-398 induces apoptosis in myeloma cells via Bcl-2 independent pathway2005

    • Author(s)
      Zang M, Abe Y, Matsushima T, Nishimura J, Nawata H, Muta K
    • Journal Title

      Leuk Lymph 46(3)

      Pages: 425-433

    • Related Report
      2005 Annual Research Report
  • [Journal Article] S Fludarabine-based conditioning used in successful bone marrow transplantation from an unrelated donor in a heavily transfused patient with severe aplastic anemia.2005

    • Author(s)
      Abe Y, Matsushima T, Tachikawa Y, Nagasawa E, Nishimura J, Nawata H, Muta K
    • Journal Title

      Int J Hematol 81(1)

      Pages: 81-82

    • Related Report
      2005 Annual Research Report
  • [Journal Article] A novel mechanism in suppression of erythropoiesis during inflammation : a crucial role of RCAS12005

    • Author(s)
      Suehiro Y, Muta K, Nakashima M, Abe Y, Shiratsuchi M, Shiokawa S, Ikuyama S, Yoshikawa Y, Watanabe T, Nishimura J
    • Journal Title

      Eur J Hematol 74

      Pages: 365-373

    • Related Report
      2005 Annual Research Report
  • [Journal Article] Molecular remission by nonmyeloablative stem cell transplantation after autologous peripheral blood stem cell transplantation in a patient with multiple myeloma2005

    • Author(s)
      Nakashima Y, Shiratsuchi M, Tani K, Abe Y, Muta K, Shiokawa S, Nishimura J
    • Journal Title

      Leukemia and Lymphoma (in press)

    • Related Report
      2004 Annual Research Report
  • [Journal Article] A novel mechanism in suppression of erythropoiesis during inflammation : a crucial role of RCAS12005

    • Author(s)
      Suehiro Y, Muta K, Nakashima M, Abe Y, Shiratsuchi M, Shiokawa S, Ikuyama S, Yoshikawa Y, Watanabe T
    • Journal Title

      European Journal of Haematology (in press)

    • Related Report
      2004 Annual Research Report
  • [Journal Article] Selective cyclooxigenase 2 inhibitor NS-398 induces apoptosis in myeloma cells via a Bcl-2 indepent pathway2005

    • Author(s)
      Zang M, Abe Y, Matsushima T, Nishimura J, Nawata H, Muta K
    • Journal Title

      Leukemia and Lymphoma 46(3)

      Pages: 425-433

    • Related Report
      2004 Annual Research Report
  • [Journal Article] Nonmyeloablative hematoopoietic stem cell transplantation for the treatment of systemic sclerosis2004

    • Author(s)
      Shiratsuchi S, Motomura S, Ikuta T, Ohno H, Shiokawa S, Nishimura J
    • Journal Title

      Blood 104(11)

    • Related Report
      2004 Annual Research Report
  • [Journal Article] Fludarabine-based conditioning used in successful bone marrow transplantation from an unrelated donor in a heavily transfused patient with severe aplastic anemia2004

    • Author(s)
      Abe Y, Matsushima T, Tachikawa Y, Nagasawa E, Nishimura J, Nawata H, Muta K
    • Journal Title

      International Journal of Hematology 81(1)

      Pages: 81-82

    • NAID

      10014282748

    • Related Report
      2004 Annual Research Report
  • [Journal Article] Successful treatment with nonmyeloablative allogeneic hematopoietic stem cell transplantation in a patient with acute myeloid leukemia complicated with pulmonary infection2004

    • Author(s)
      Choi I, Abe Y, Otsuka R, Matsushima T, Y Tachikawa, Nagasawa E, Nishimura J, Inaba S, Nawata H, Muta K
    • Journal Title

      International Journal of Hematology 78(1)

      Pages: 92-94

    • NAID

      50000391301

    • Related Report
      2004 Annual Research Report
  • [Book] 慢性骨髄性白血病、Year Notes Selected Article2005

    • Author(s)
      西村純二(分担、監修)
    • Publisher
      Medic Media社
    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2005 Final Research Report Summary
  • [Book] 慢性骨髄性白血病、Year Note Selected Article2005

    • Author(s)
      西村純二(分担、監修)
    • Publisher
      Medic Media社
    • Related Report
      2005 Annual Research Report

URL: 

Published: 2004-04-01   Modified: 2016-04-21  

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