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

助川 慎一郎; 坂田（柳元） 麻実子; 松岡 亮太; 百瀬 春佳; 清木 祐介; 野口 雅之; 中村 直哉; 渡辺 玲; 藤本 学; 横山 泰久; 錦井 秀和; 加藤 貴康; 日下部 学; 栗田 尚樹; 小原 直; 長谷川 雄一; 千葉 滋

doi:10.11406/rinketsu.59.2567

Case Reports

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

Shinichiro SUKEGAWA, Mamiko SAKATA-YANAGIMOTO, Ryota MATSUOKA, Haruka MOMOSE, Yusuke KIYOKI, Masayuki NOGUCHI, Naoya NAKAMURA, Rei WATANABE, Manabu FUJIMOTO, Yasuhisa YOKOYAMA, Hidekazu NISHIKII, Takayasu KATO, Manabu KUSAKABE, Naoki KURITA, Naoshi OBARA, Yuichi HASEGAWA, Shigeru CHIBA

Author information

Shinichiro SUKEGAWA
Department of Hematology, University of Tsukuba Hospital
Mamiko SAKATA-YANAGIMOTO
Department of Hematology, University of Tsukuba Hospital
Department of Hematology, Faculty of Medicine, University of Tsukuba
Ryota MATSUOKA
Department of Pathology, University of Tsukuba Hospital
Haruka MOMOSE
Department of Hematology, University of Tsukuba Hospital
Yusuke KIYOKI
Department of Hematology, University of Tsukuba Hospital
Masayuki NOGUCHI
Department of Pathology, University of Tsukuba Hospital
Naoya NAKAMURA
Department of Pathology, Tokai University School of Medicine
Rei WATANABE
Department of Dermatology, Faculty of Medicine, University of Tsukuba
Manabu FUJIMOTO
Department of Dermatology, Faculty of Medicine, University of Tsukuba
Yasuhisa YOKOYAMA
Department of Hematology, University of Tsukuba Hospital
Department of Hematology, Faculty of Medicine, University of Tsukuba
Hidekazu NISHIKII
Department of Hematology, University of Tsukuba Hospital
Department of Hematology, Faculty of Medicine, University of Tsukuba
Takayasu KATO
Department of Hematology, University of Tsukuba Hospital
Department of Hematology, Faculty of Medicine, University of Tsukuba
Manabu KUSAKABE
Department of Hematology, University of Tsukuba Hospital
Department of Hematology, Faculty of Medicine, University of Tsukuba
Naoki KURITA
Department of Hematology, University of Tsukuba Hospital
Department of Hematology, Faculty of Medicine, University of Tsukuba
Naoshi OBARA
Department of Hematology, University of Tsukuba Hospital
Department of Hematology, Faculty of Medicine, University of Tsukuba
Yuichi HASEGAWA
Department of Hematology, University of Tsukuba Hospital
Department of Hematology, Faculty of Medicine, University of Tsukuba
Shigeru CHIBA
Department of Hematology, University of Tsukuba Hospital
Department of Hematology, Faculty of Medicine, University of Tsukuba

Keywords: BPDCN, CMML, Azacitidine

JOURNAL RESTRICTED ACCESS

2018 Volume 59 Issue 12 Pages 2567-2573

DOI https://doi.org/10.11406/rinketsu.59.2567

Details

Abstract

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.

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