Blastic plasmacytoid dendritic cell neoplasm:clinicopathological and molecular genetic characteristics
TONG Xing
GUO Ling-chuan
Abstract：Objective To explore the clinicopathological and molecular genetic characteristics of blastic plasmacytoid dendritic cell neoplasm(BPDCN)and to improve the understanding about it.Methods The clinicopathologic data of 8 BPDCN cases were analyzed retrospectively and the related literatures were reviewed.Results There were 5 males and 3 females.The tumor cells were diffusely infiltrating,medium in size,uniform in morphology and showed a mother cell pattern.The mitoses were visible.The result of immunophenotype showed that all 8 cases were positive for CD123,CD43 and CD4,CD56 positive in 7 cases,TdT positive in 2 cases,the Ki-67 proliferation index was around 35%-95%.By targeted next generation sequencing,DNMT3A Arg882His mutation was found in one case and CSMD1 N191K mutation,a germline mutation,was found in another.Conclusion BPDCN is a rare and aggressive hematologic malignancy with poor clinical outcomes.It has various clinical manifestations,often involving skin,lymph nodes,as well as bone marrow.Its diagnosis predominantly depends on clinical manifestations and the result of pathological examination.Patients with skin solitary lesion may benefit from surgical resection.
Keywords：Blastic plasmacytoid dendritic cell neoplasmclinicopathologysecond generation sequencinglymphoid and hematopoietic neoplasm
Publication Date：2023-12-28
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：5( 1071-1074,1079 )