Blastic plasmacytoid dendritic cell neoplasm:a clinicopathologic study of four cases
Objective To explore the clinicopathological features,immunophenotyping,differential diagnosis,treatment and prognosis of blastic plasmacytoid dendritic cell neoplasm(BPDCN).Methods The clinical manifestations,Imaging,histopathology,immunophenotypic features and gene sequencing results of 4 patients with BPDCN were analyzed retrospectively.The related literature was reviewed.Results Among the 4 patients,3 were male and 1 was female.The age of onset ranged from 17 to 59 years,with a median age of 52 years.4 cases had skin invasion as the first symptom,2 cases had lymph node metastasis,3 cases had bone marrow involvement,and 1 case had central and peripheral nervous system invasion.Tumor cell specific immune markers CD4,CD56,CD43 and BCL-2 were all positive,and CD123 in 3 patients.Ki-67 index was 20％-90％.EBER in situ hybridization was negative.In one case,there was no obvious chromosome abnormality,and the mutation analysis of 74 genes in the medullary system was as follows:ASXL1 24.8％,TET2 20.6％.The clonal analysis of T cell receptor gene rearrangement was all negative.Conclusion BPDCN is a rare and highly aggressive hematopoietic neoplasm with rapid progress and the high mortality.The positive expression of CD4,CD56,CD43 and BCL-2 is a significant immunephenotype and does not express myeloid cells or B,T lymphocyte immune markers.In addition to pathological morphology,comprehensive analysis such as clinical manifestations,immunophenotypes,and laboratory tests should also be combined during diagnosis.If necessary,molecular genetic testing should be used to assist in diagnosis to avoid misdiagnosis and missed diagnosis.
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