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Journal Of Modern Oncology[ISSN:1672-4992/CN:61-1415/R]

Issue:

2022 04

Page:

709-713

Research Field:

Publishing date:

Info

Title:

Research progress in pathogenesis,diagnosis and treatment of blastic plasmacytoid dendritic cell neoplasm

Author(s):

WANG Ying¹; 2; MA Yinjuan²; XU Wenjing²; YAO Hao¹; 3; BAI Hai¹; 2

1.Gansu University of Chinese Medicine,Gansu Lanzhou 730000,China;2.Deparment of Hematology,940 Hospital of PLA Joint Service Support Force,Gansu Lanzhou 730050,China;3.Deparment of Hematology,the General Hospital of Western Theater Command PLA,Sichuan Chengdu 610083,China.

Keywords:

blastic plasmacytoid dendritic cell neoplasm; plasmacytoid dendritic cell; leukemia; HSCT; targeted therapies

PACS:

R733

DOI:

10.3969/j.issn.1672-4992.2022.04.032

Abstract:

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological clonal malignant tumor derived from a precursor of dendritic cells,often involving the skin,bone marrow and lymph nodes,with a poor prognosis.BPDCN is often associated with complex karyotypes,frequent deletions of tumor suppressor genes,and mutations affecting DNA methylation or chromatin remodeling pathways.At present,there is no unified standard for the treatment of BPDCN,and the regimen mainly adopts acute myeloid leukemia,acute lymphoblastic leukemia and lymphoma.It has a high response to chemotherapy,but the median eventless survival time is usually short,generally less than two years.Allogeneic hematopoietic stem cell transplantation can improve the prognosis of BPDCN,but the recurrence rate is still very high.In 2018,SL-401 was approved as the first targeted therapy for BPDCN patients 2 years and older.With the introduction of new targeted therapies,these data on new drugs that target epigenetic changes,specific signaling pathways or neoplasm cells expressing antigens show promise and pave the way for prospective studies using targeted therapies.In this paper,the pathogenesis,diagnosis and treatment of BPDCN are reviewed,and new targeted therapies are discussed.

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