Two Cases of Blastic Plasmacytoid Dendritic Cell Neoplasm

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  • SHIRASE Haruna
    Department of Dermatology, School of Medicine, University of the Ryukyus
  • YAMAGUCHI Sayaka
    Department of Dermatology, School of Medicine, University of the Ryukyus
  • TAIRA Kiyohito
    Department of Dermatology, School of Medicine, University of the Ryukyus
  • HAYASHI Kentarou
    Department of Dermatology, School of Medicine, University of the Ryukyus
  • KARIYA Yoshiyuki
    Department of Dermatology, School of Medicine, University of the Ryukyus
  • YAMAMOTO Yuichi
    Department of Dermatology, School of Medicine, University of the Ryukyus
  • TAKAHASHI Kenzo
    Department of Dermatology, School of Medicine, University of the Ryukyus
  • UEZATO Hiroshi
    Department of Dermatology, School of Medicine, University of the Ryukyus

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Other Title
  • Blastic Plasmacytoid Dendritic Cell Neoplasm：芽球性形質細胞様樹状細胞腫瘍の 2 例
  • 症例 Blastic Plasmacytoid Dendritic Cell Neoplasm : 芽球性形質細胞様樹状細胞腫瘍の2例
  • ショウレイ Blastic Plasmacytoid Dendritic Cell Neoplasm : 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 ノ 2レイ

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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a recently classified, rare and aggressive hematodermic neoplasm. We report here two elderly cases of BPDCN with poor prognoses. An 85-year-old man had a skin tumor on his right thigh, and then multiple erythemas and nodules appeared on the whole body. A histological analysis revealed a dense proliferation of atypical mononuclear cells from the dermis to subcutaneous tissue. The tumor cells were CD4, CD7, CD56, CD123, TCL-1 positive, and CD20, CD45RO, EBER negative, so the tumor was diagnosed as BPDCN. He developed leukemia 1 week after the first visit and died 1 month later. A 77-year-old man had a skin tumor on his back and then multiple erythemas appeared on the entire body. A histological analysis revealed intense proliferation of atypical cells, which showed the same surface markers as those of case 1, thereby establishing the diagnosis as BPDCN. Although he underwent chemotherapy with THP-COP and low dosage etoposide, he died of leukemia 10 months after his first visit to our hospital. BPDCN is characterized by the clonal expansion of precursor plasmacytoid dendritic cells, and the cytological analysis represented by CD56, CD123, and T cell leukemic/lymphoma-1 is the key diagnostic tool.

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