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A Journal on Dermatology
Esperienze Dermatologiche 2016 March;18(1):16-20
Blastic plasmacytoid dendritic cell neoplasm: case series and a brief review of literature
Nicola DI MEO 1, Giuseppe STINCO 2, Sara TREVISINI 1, Cinzia BULIGAN 2, Eugenio LEONARDO 3, Giusto TREVISAN 1
1 Department of Dermatology and Venereology, University of Trieste, Ospedale Maggiore of Trieste, Trieste Italy; 2 Department of Clinical and Experimental Medicine, University of Udine, Institute of Dermatology, Academic Hospital of Udine, Udine, Italy; 3 Department of Pathology, Ospedali Riuniti di Trieste University Hospital, Trieste, Italy
Blastic plasmacytoid dendritic cell neoplasm is a very aggressive disease which has been classified by the World Health Organization among “acute myeloid leukemia and related precursor neoplasms” in 2008. In early stages the skin is almost always the first affected site. Histological exam and immunophenotype are mandatory and necessary for the right diagnosis; its management is still a challenge. It is rare (only 0.7% of cutaneous leukemia in western countries). Recently five patients affected by blastic plasmacytoid dendritic cell neoplasm came to our attention. They had different clinical manifestations at first visit and different behaviour at the beginning, but they had the same immunohistochemical pattern and the same poor prognosis.