Conflicts of interest None declared.
Blastic plasmacytoid dendritic cell neoplasm: a report of four cases and review of the literature
Article first published online: 27 MAR 2012
© 2012 The Author. Journal of the European Academy of Dermatology and Venereology © 2012 European Academy of Dermatology and Venereology
Journal of the European Academy of Dermatology and Venereology
Volume 27, Issue 9, pages 1176–1181, September 2013
How to Cite
Borchiellini, D., Ghibaudo, N., Mounier, N., Del Giudice, P., Quinsat, D., Ticchioni, M., Perrin, C., Cardot Leccia, N. and Lacour, J.P. (2013), Blastic plasmacytoid dendritic cell neoplasm: a report of four cases and review of the literature. Journal of the European Academy of Dermatology and Venereology, 27: 1176–1181. doi: 10.1111/j.1468-3083.2012.04503.x
- Issue published online: 28 JUL 2013
- Article first published online: 27 MAR 2012
- Received: 9 August 2011; Accepted: 16 February 2012
Background Blastic plasmacytoid dendritic cell neoplasm (BPDCN), formerly known as agranular CD4+/CD56+ haematodermic neoplasm (CD4/CD56 HN), is a rare distinct form of lymphoma-like entity known of dermatologists because of its marked predilection for cutaneous involvement, and its aggressive behaviour. Moreover, the association or the evolution to an acute leukaemia entity that still expresses CD4 and CD56 markers is almost systematic. This new described entity of ‘CD4+/CD56+ leukaemia’ or ‘leukaemia of plasmacytoid dendritic cell lineage’ has a poor prognostic and may lead to include haematopoietic stem cell transplantation in the treatment strategy as early as possible.
Report of cases We report here four cases presenting with skin lesions and haematological signs. One of the patients underwent allogeneic stem cell transplantation, with a relapse-free survival of 40 months. We discuss the diagnosis features as well as the treatment options.
Conclusion A collaborative work between dermatologists and onco-haematologists is essential to give patients the best chance of complete and long-term response.