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Rinsho Ketsueki. 2018;59(12):2567-2573. doi: 10.11406/rinketsu.59.2567.

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

[Article in Japanese]

Author information

Department of Hematology, University of Tsukuba Hospital.
Department of Hematology, Faculty of Medicine, University of Tsukuba.
Department of Pathology, University of Tsukuba Hospital.
Department of Pathology, Tokai University School of Medicine.
Department of Dermatology, Faculty of Medicine, University of Tsukuba.


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.


Azacitidine; BPDCN; CMML


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