Blastic plasmacytoid dendritic cell neoplasm
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Synopsis
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy derived from plasmacytoid dendritic cells. The incidence is unknown due to its rarity; however, among the cutaneous lymphomas, it represents less than 1% of cases. Most reported cases are in adults, with a median age of 65-67 years at diagnosis.
BPDCN usually has an initial cutaneous presentation with or without bone marrow involvement and rarely presents as a leukemia. The skin manifestations are red, brown, or violaceous papules, plaques, nodules, or tumors that may be localized or widespread. The presence of eruptive cutaneous lesions portends a worse prognosis. Extracutaneous involvement has been reported in tonsils, paranasal cavities, lungs, eyes, and the central nervous system. Patients may have cytopenias, lymphadenopathy, and/or splenomegaly as well.
BPDCN usually has an initial cutaneous presentation with or without bone marrow involvement and rarely presents as a leukemia. The skin manifestations are red, brown, or violaceous papules, plaques, nodules, or tumors that may be localized or widespread. The presence of eruptive cutaneous lesions portends a worse prognosis. Extracutaneous involvement has been reported in tonsils, paranasal cavities, lungs, eyes, and the central nervous system. Patients may have cytopenias, lymphadenopathy, and/or splenomegaly as well.
Codes
ICD10CM:
C86.4 – Blastic NK-cell lymphoma
SNOMEDCT:
445105005 – Blastic plasmacytoid dendritic cell neoplasm
C86.4 – Blastic NK-cell lymphoma
SNOMEDCT:
445105005 – Blastic plasmacytoid dendritic cell neoplasm
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Last Reviewed:09/10/2019
Last Updated:09/18/2019
Last Updated:09/18/2019