Plasmacytoid dendritic cell neoplasms

• Published in: Blood research Vol. 58; no. S1; pp. 90 - S95
• Main Authors: Lee, Yoo Jin, Kim, Youjin, Park, Sang Hyuk, Jo, Jae-Cheol
• Format: Journal Article
• Language: English
• Published: Korea (South) Korean Society of Hematology; Korean Society of Blood and Marrow Transplantation; Korean Society of Pediatric Hematology-Oncology; Korean Society on Thrombosis and Hemostasis 30.04.2023; 대한혈액학회
• Subjects: Review; 병리학; Mature plasmacytoid dendritic cell proliferation; Plasmacytoid dendritic cell neoplasm; Blastic plasmacytoid dendritic cell neoplasm
• ISSN: 2287-979X; 2288-0011
• DOI: 10.5045/br.2023.2023052

Plasmacytoid dendritic cells (pDCs) are type I interferon-producing cells that modulate immune responses. There are two types of pDC neoplasms: 1) mature pDC proliferation (MPDCP) associated with myeloid neoplasm and 2) blastic pDC neoplasm (BPDCN). MPDCP is a clonal expansion of mature pDCs that is predominantly associated with chronic myelomonocytic leukemia. In contrast, BPDCN is a clinically aggressive myeloid malignancy involving the skin, bone marrow, lymphatic organs, and central nervous system. There are various types of skin lesions, ranging from solitary brown or violaceous to disseminated cutaneous lesions, which often spread throughout the body. The expression of CD4, CD56, CD123, and pDC markers (TCL-1, TCF4, CD303, and CD304, etc.) are typical immunophenotype of BPDCN. Historically, BPDCN treatment has been based on acute leukemia regimens and allogeneic hematopoietic cell transplantation in selected patients. Recent advances in molecular biology and genetics have led to the development of targeted agents, such as tagraxofusp (a recombinant fusion protein targeting CD123), anti-CD123 CAR-T cells, XmAb14045, and IMGN632. Lastly, this review provides a comprehensive overview of pDC neoplasms.