Haploidentical transplantation from a related donor as first-line treatment for fulminant aplastic anemia

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  • MAKISHIMA Kenichi
    Department of Hematology, University of Tsukuba Hospital
  • OBARA Naoshi
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Faculty of Medicine, University of Tsukuba
  • SAKAMOTO Tatsuhiro
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Faculty of Medicine, University of Tsukuba
  • KATO Takayasu
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Faculty of Medicine, University of Tsukuba
  • KUSAKABE Manabu
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Faculty of Medicine, University of Tsukuba
  • NISHIKII Hidekazu
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Faculty of Medicine, University of Tsukuba
  • KURITA Naoki
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Faculty of Medicine, University of Tsukuba
  • YOKOYAMA Yasuhisa
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Faculty of Medicine, University of Tsukuba
  • SAKATA-YANAGIMOTO Mamiko
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Faculty of Medicine, University of Tsukuba
  • HASEGAWA Yuichi
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Faculty of Medicine, University of Tsukuba
  • CHIBA Shigeru
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Faculty of Medicine, University of Tsukuba

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Other Title
  • 初回治療としてHLA半合致血縁ドナーから同種造血幹細胞移植を施行した劇症型再生不良性貧血
  • 症例報告 初回治療としてHLA半合致血縁ドナーから同種造血幹細胞移植を施行した劇症型再生不良性貧血
  • ショウレイ ホウコク ショカイ チリョウ ト シテ HLA ハンガッチ ケツエン ドナー カラ ドウシュ ゾウケツ カンサイボウ イショク オ シコウ シタ ゲキショウガタ サイセイ フリョウセイ ヒンケツ

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Abstract

<p>Immunosuppressive therapy (IST) is the first-line treatment for young patients with severe aplastic anemia (AA) when a human leucocyte antigen (HLA)-matched related donor (MRD) is unavailable. Fulminant AA (FAA) is defined as AA with a complete absence of neutrophils at presentation and no response to granulocyte-colony stimulating factor (G-CSF) treatment. Here we report a 38-year-old male FAA patient who underwent allogeneic stem cell transplantation from an HLA haplotype-mismatched donor as first-line treatment. The patient had no remarkable disease history and was referred to our hospital because of a peritonsillar abscess and severe pancytopenia. Bone marrow biopsy revealed marked hypocellularity without dysplasia. His neutrophil count remained 0.0×109/l following G-CSF administration, and he was diagnosed with FAA. His siblings were not MRDs, but his sister had haploidentical HLAs. After administering a conditioning regimen, the patient received a transplant of peripheral blood stem cells donated by his sister. Neutrophil engraftment was observed on post-transplant day 16, and he experienced acute graft-versus-host disease (grade I, skin stage 1), but no other complications were observed. Hematopoietic stem cell transplantation from an HLA haplotype-mismatched related donor may be a viable option for first-line treatment of FAA when an MRD is unavailable.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 61 (10), 1464-1468, 2020

    The Japanese Society of Hematology

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