Blastic plasmacytoid dendritic cell neoplasm – pro
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, clinically aggressive hematologic malignancy that most commonly manifests as cutaneous lesions with or without bone marrow involvement and leukemic dissemination. It is an aggressive hematologic malignancy derived from plasmacytoid dendritic cells that typically involves the skin and rapidly progresses to a leukemia phase. Despite being initially responsive to intensive combination chemotherapy, most patients relapse and succumb to their disease.The rarity of this disease does not enable prospective clinical trials to identify the better therapeutic strategy, which, at present, is based on clinicians’ experience. Many clinicians use an AML like regimen, such as idarubicin and cytarrabine, and go on to an allogeneic stem cell transplantation, which is case reports appears to be somewhat successful.
AUPagano L, Valentini CG, Pulsoni A, Fisogni S, Carluccio P, Mannelli F, Lunghi M, Pica G, Onida F, Cattaneo C, Piccaluga PP, Di Bona E, Todisco E, Musto P, Spadea A, D’Arco A, Pileri S, Leone G, Amadori S, Facchetti F, GIMEMA-ALWP (Gruppo Italiano Malattie EMatologiche dell’Adulto, Acute Leukemia Working Party). Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation: an Italian multicenter study. SOHaematologica. 2013 Feb;98(2):239-46. Epub 2012 Oct 12.
Rauh MJ, Rahman F, Good D, et al. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation, lacking cutaneous involvement: Case series and literature review. Blastic dendritic cell neoplasm. Br J Haematol 2011; 153:413.
Leuk Res 2012; 36:81.
Roos-Weil D, Dietrich S, Boumendil A, et al. Stem cell transplantation can provide durable disease control in blastic plasmacytoid dendritic cell neoplasm: a retrospective study from the European Group for Blood and Marrow Transplantation. Blood 2013; 121:440.