ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferencesView all Images (5)
Blastic plasmacytoid dendritic cell neoplasm
Other Resources UpToDate PubMed

Blastic plasmacytoid dendritic cell neoplasm

Contributors: Nina Haghi MD, Paritosh Prasad MD, Susan Burgin MD
Other Resources UpToDate PubMed

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.

Codes

ICD10CM:
C86.4 – Blastic NK-cell lymphoma

SNOMEDCT:
445105005 – Blastic plasmacytoid dendritic cell neoplasm

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed:09/11/2019
Last Updated:09/19/2019
Copyright © 2021 VisualDx®. All rights reserved.
Blastic plasmacytoid dendritic cell neoplasm
Blastic plasmacytoid dendritic cell neoplasm : Lymphadenopathy, Splenomegaly
Copyright © 2021 VisualDx®. All rights reserved.