from Section IV - Precursor Hematopoietic Neoplasms and Related Neoplasms
Published online by Cambridge University Press: 25 November 2023
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive malignancy that is notable for frequently presenting with skin lesions and for its unfavorable prognosis. The revised fourth edition of the World Health Organization classification places BPDCN in a separate, stand-alone chapter between acute myeloid leukemia and acute leukemias of ambiguous lineage in recognition of its distinct lineage and aggressive clinical behavior. [1]. Knowledge of the cell of origin and the increasing availability of markers specific for plasmacytoid dendritic cells has recently allowed the recognition of heterogeneity in the clinical presentation and immunophenotype of BPDCN [2].
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