Blastic plasmacytoid dendritic cell neoplasm accompanied by chronic myelomonocytic leukemia successfully treated with azacitidine

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  • SUKEGAWA Shinichiro
    Department of Hematology, University of Tsukuba Hospital
  • SAKATA-YANAGIMOTO Mamiko
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Faculty of Medicine, University of Tsukuba
  • MATSUOKA Ryota
    Department of Pathology, University of Tsukuba Hospital
  • MOMOSE Haruka
    Department of Hematology, University of Tsukuba Hospital
  • KIYOKI Yusuke
    Department of Hematology, University of Tsukuba Hospital
  • NOGUCHI Masayuki
    Department of Pathology, University of Tsukuba Hospital
  • NAKAMURA Naoya
    Department of Pathology, Tokai University School of Medicine
  • WATANABE Rei
    Department of Dermatology, Faculty of Medicine, University of Tsukuba
  • FUJIMOTO Manabu
    Department of Dermatology, Faculty of Medicine, University of Tsukuba
  • YOKOYAMA Yasuhisa
    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
  • 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
  • KURITA Naoki
    Department of Hematology, University of Tsukuba Hospital Department of Hematology, Faculty of Medicine, University of Tsukuba
  • OBARA Naoshi
    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

Bibliographic Information

Other Title
  • アザシチジンが有効であった慢性骨髄単球性白血病合併の芽球性形質細胞様樹状細胞腫瘍
  • 症例報告 アザシチジンが有効であった慢性骨髄単球性白血病合併の芽球性形質細胞様樹状細胞腫瘍
  • ショウレイ ホウコク アザシチジン ガ ユウコウ デ アッタ マンセイ コツズイタンキュウセイ ハッケツビョウ ガッペイ ノ ガキュウセイ ケイシツ サイボウ ヨウジュジョウ サイボウ シュヨウ

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Abstract

<p>Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare disease that develops with a skin lesion and is often accompanied by leukemic transformation. The normal counterparts of BPDCN tumor cells are progenitors of plasmacytoid dendritic cells, whereas the origins are thought to be hematopoietic stem cells. Approximately 10%-20% of BPDCN patients develop other hematologic malignancies, including chronic myelomonocytic leukemia (CMML). Mutations in epigenetic regulators are frequently observed in both BPDCN and CMML tumors. Azacitidine, a drug that targets epigenetic dysregulation, is known to be an effective treatment for CMML. However, it has been used in few BPDCN patients. Here, we report a BPDCN patient with skin lesions, bone marrow infiltration, and lymphadenopathy. CMML also developed during the course of BPDCN. Azacitidine had positive effects on CMML; however, BPDCN aggressively relapsed during treatment. Two TET2 mutations were found in both BPDCN and CMML tumors; one of which was commonly identified in both tumors.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 59 (12), 2567-2573, 2018

    The Japanese Society of Hematology

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