Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a newly identified, very rare, highly aggressive hematopoietic neoplasm, primarily found in elderly males. They typically present in the form of skin involvement with a high frequency of lymph node and bone marrow involvement. BPDCN has a very poor prognosis, with no consensus on a widely accepted treatment modality. Here we present a very young patient with BPDCN, who presented with generalized lymphadenopathy, skin involvement, and leukemic blasts in the bone marrow. She was treated with high-risk acute lymphocytic leukemia protocol, followed by allogeneic hematopoietic stem-cell transplantation, and has been in clinical remission for 12 months.

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http://dx.doi.org/10.1097/MPH.0000000000001999DOI Listing

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Department of Hematology, Hebei General Hospital,Shijiazhuang 050051,China.

Blastic plasmacytoid dendritic cell neoplasm(BPDCN)is an extremely rare invasive tumor with poor prognosis.The common clinical manifestations of BPDCN include skin injury,bone marrow involvement,and tumor cell spread.BPDCN is often misdiagnosed as other diseases and its diagnosis often requires a combination of clinical manifestations,imaging,histology,and immunophenotyping.

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  • Chronic myelomonocytic leukemia (CMML) is a complex blood cell disorder that combines poorly functioning and overproducing blood cells, leading to various symptoms.
  • The case study focuses on a 71-year-old man with severe CMML who experienced skin nodules that improved with treatment but later recurred, while avoiding progression to acute leukemia.
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[Clinical and Pathological Features of Blastic Plasmacytoid Dendritic Cell Neoplasm].

Zhongguo Shi Yan Xue Ye Xue Za Zhi

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Department of Laboratory Medicine, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China.

Objective: To summarize and analyze the clinical features of blastic plasmacytoid dendritic cell neoplasm (BPDCN), so as to enhance the understanding of this disease.

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Background/aim: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly aggressive hematologic cancer which is difficult to diagnose and has a lot of overlapping features with other diseases, particularly acute myeloid leukemia (AML). BPDCN shares several immunophenotypic markers with AML, such as CD4, CD56, CD123, and HLA-DR, stating the importance of having extending panel of specific immunohistochemical (IHC) markers.

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