Aggressive NK-cell leukemia with sustained relapse-free survival after allogeneic peripheral blood stem cell transplantation

  • INO Kazuko
    Department of Internal Medicine, Matsusaka Chuo General Hospital Department of Hematology and Oncology, Mie University Graduate School of Medicine
  • MASUYA Masahiro
    Department of Hematology and Oncology, Mie University Graduate School of Medicine
  • NAKAMORI Yoshiki
    Department of Internal Medicine, Matsusaka Chuo General Hospital
  • SUZUKI Kei
    Department of Hematology and Oncology, Mie University Graduate School of Medicine
  • MIZUTANI Minoru
    Department of Internal Medicine, Matsusaka Chuo General Hospital
  • SEKINE Takao
    Department of Internal Medicine, Matsusaka Chuo General Hospital
  • YAMAGUCHI Motoko
    Department of Hematology and Oncology, Mie University Graduate School of Medicine
  • NAKASE Kazunori
    Department of Hematology and Oncology, Mie University Graduate School of Medicine
  • KAIDA Katsuji
    Division of Hematology, Department of Internal Medicine, Hyogo College of Medicine
  • OGAWA Hiroyasu
    Division of Hematology, Department of Internal Medicine, Hyogo College of Medicine
  • KATAYAMA Naoyuki
    Department of Hematology and Oncology, Mie University Graduate School of Medicine

Bibliographic Information

Other Title
  • 同種末梢血幹細胞移植後長期間無再発生存中のアグレッシブNK細胞白血病
  • 症例報告 同種末梢血幹細胞移植後長期間無再発生存中のアグレッシブNK細胞白血病
  • ショウレイ ホウコク ドウシュ マッショウケツ カンサイボウ イショク ゴ チョウキカン ムサイハツ セイゾン チュウ ノ アグレッシブ NK サイボウ ハッケツビョウ

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Abstract

A 46-year-old Japanese man was admitted to our hospital because of prolonged fever. Laboratory examination demonstrated leukopenia, thrombocytopenia, marked liver dysfunction, and elevation of serum ferritin. A bone marrow examination showed several hemophagocytic macrophages, and a diagnosis of hemophagocytic syndrome was made. He was treated using HLH-94 protocol, and his clinical symptoms and laboratory data were rapidly improved. After 5 weeks, fever and liver dysfunction reappeared. A repeat bone marrow examination demonstrated that 28.4% of marrow nucleated cells were atypical lymphocytes, which were positive for CD2, CD7, CD16, CD56, and HLA-DR. Clonality of these proliferating NK cells was confirmed by an analysis of EB virus terminal repeat sequence and cytogenetic analysis, and final diagnosis of aggressive NK-cell leukemia was made. After induction chemotherapy consisting of dexamethasone, etoposide, ifosfamide, and L-asparaginase, the patient achieved partial remission. He received allogeneic peripheral blood stem cell transplantation from his one locus mismatched son, and is alive with no evidence of disease 20 months after transplantation.

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 51 (4), 258-263, 2010

    The Japanese Society of Hematology

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