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Chapter 14 - Blastic Plasmacytoid Dendritic Cell Neoplasm

from Section IV - Precursor Hematopoietic Neoplasms and Related Neoplasms

Published online by Cambridge University Press:  25 November 2023

By
Stephanie D. Schniederjan ,
Aurelia Meloni-Ehrig ,
Caroline An  and
Kristian T. Schafernak
Edited by
Silvia Tse Bunting ,
Xiayuan Liang ,
Michele E. Paessler  and
Satheesh Chonat
Silvia Tse Bunting
Affiliation:
Cleveland Clinic Florida Weston
Xiayuan Liang
Affiliation:
University of Colorado
Michele E. Paessler
Affiliation:
University of Pennsylvania School of Medicine
Satheesh Chonat
Affiliation:
Emory University, Atlanta
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Summary

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].

Type
Chapter
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Atlas of Pediatric Hematopathology , pp. 140 - 146
Publisher: Cambridge University Press
Print publication year: 2023

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References

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