Blastic plasmacytoid dendritic cell neoplasm and the related diseases: Development of diagnostic methods and elucidation of pathogenesis

2021 Fiscal Year Final Research Report

• Project/Area Number: 19K16596
• Research Category: Grant-in-Aid for Early-Career Scientists
• Allocation Type: Multi-year Fund
• Review Section: Basic Section 49020:Human pathology-related
• Research Institution: Japanese Foundation for Cancer Research
• Principal Investigator: SAKAMOTO Kana 公益財団法人がん研究会, がん研究所 分子標的病理プロジェクト, 研究員 (50778008)
• Project Period (FY): 2019-04-01 – 2022-03-31
• Keywords: 芽球性形質細胞様樹状細胞腫瘍

Outline of Final Research Achievements

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematopoietic neoplasm with a poor prognosis. Due to the rarity of the disease and the delayed establishment as a disease entity, diagnostic criteria have not been fully established. Differentiating BPDCN from acute myeloid leukemia is particularly challenging. In this study, we developed a simple and highly accurate diagnostic method using immunohistochemistry based on the studies of a large group of cases diagnosed or suspected to have BPDCN at multiple institutions in Japan.

Free Research Field

人体病理学

Academic Significance and Societal Importance of the Research Achievements

BPDCNは、病態にいまだ不明な点が多く、予後不良であることからその解明が求められている。今回の検討から、既存の診断法におけるピットフォールとなりうる点が明らかになり、多数例での検討に担保された新たな診断法を提唱することができた。正確な診断は適切な治療、病態研究にあたり必須のものであり、今後のBPDCN診療の発展に資するものと考えられる。