Blastic neoplasia of plasmacytoid dendritic cells

Last Updated: 2023-07-07

Author(s): Anzengruber F., Navarini A.

ICD11: 2A60.5

Petrella 1999

Aggressive neoplasia, which primarily starts on the skin and becomes generalised leukaemic in the course of the disease.

  • Rare
  • Peak in frequency: 70-80 y

  • Exanthematous, reddish-brown, asymptomatic plaques and nodules on the trunk and head
  • Involvement of CNS and bone marrow is typical

The average survival time is 12-14 months.

  1. Angelot-Delettre, F., et al., In vivo and in vitro sensitivity of blastic plasmacytoid dendritic cell neoplasm to SL-401, an interleukin-3 receptor targeted biologic agent. Haematologica, 2015. 100(2): p. 223-30.
  2. Laribi, K., et al., Blastic Plasmacytoid Dendritic Cell Neoplasm: From Origin of the Cell to Targeted Therapies. Biol Blood Marrow Transplant, 2016.
  3. Kim, J.H., et al., Blastic Plasmacytoid Dendritic Cell Neoplasm: Analysis of Clinicopathological Feature and Treatment Outcome of Seven Cases. Ann Dermatol, 2015. 27(6): p. 727-37.
  4. Facchetti, F., et al., Neoplasms derived from plasmacytoid dendritic cells. Mod Pathol, 2016. 29(2): p. 98-111.