A Case Series of Blastic Plasmacytoid Dendritic Cell Neoplasia

Blastic plasmacytoid dendritic cell neoplasm (BPDCN), an extremely rare and aggressive tumor, derives from plasmacytoid dendritic cell precursors and is characterized by CD4 and CD56 positivity accompanied by the expression of isolated myeloid, B- or T-cell lineage markers. Despite the recent introd...

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Bibliographic Details
Published in:Translational medicine @ UniSa 2020-10, Vol.23 (4), p.63-66
Main Authors: Serio, B, Giudice, V, D'Addona, M, Guariglia, R, Gorrese, M, Bertolini, A, D'Alto, F, Cuffa, B, Pellegrino, D, Langella, M, Selleri, C
Format: Article
Language:English
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Summary:Blastic plasmacytoid dendritic cell neoplasm (BPDCN), an extremely rare and aggressive tumor, derives from plasmacytoid dendritic cell precursors and is characterized by CD4 and CD56 positivity accompanied by the expression of isolated myeloid, B- or T-cell lineage markers. Despite the recent introduction of specific targeted therapies, prognosis is still poor with a median overall survival of one year, and allogeneic bone marrow transplantation remains the only curative treatment in eligible patients. In this series, we described two cases of adult BPDCN treated with high dose cytarabine and methotrexate and autologous hematopoietic stem cell transplantation, or fludarabine, cytarabine, and idarubicin achieving the first a complete lasting remission, while the second only a transient improvement in skin lesions.
ISSN:2239-9747
2239-9747
DOI:10.37825/2239-9747.1012