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Blastic plasmacytoid dendritic cell neoplasm: From the origin of the cell to targeted therapies; Review

Abstract : Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy with an aggressive clinical course. It is grouped with acute myeloid leukemia–related precursor neoplasms in the 2008 World Health Organization classification. Most patients with BPDCN have skin lesions at diagnosis and subsequent or simultaneous involvement of the bone marrow, peripheral blood, and lymph nodes. Patients usually respond to initial chemotherapy but often relapse. Stem cell transplantation may improve survival. This neoplasm is derived from precursors of plasmacytoid dendritic cells and is characterized by the coexpression of the immunophenotypic markers CD4, CD56, CD123, blood dendritic cell antigen-2, blood dendritic cell antigen-4, CD2AP, and lineage−. Atypical immunophenotype expression may be present, making diagnosis difficult. BPDCN is often associated with a complex karyotype, frequent deletions of tumor suppressor genes, and mutations affecting either the DNA methylation or chromatin remodeling pathways. A better understanding of the etiology and pathophysiology of this neoplasm could open the way to new therapies targeting specific signaling pathways or involving epigenetics.
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https://hal-univ-paris10.archives-ouvertes.fr/hal-01389961
Contributor : Alix Baugier de Materre <>
Submitted on : Sunday, October 30, 2016 - 10:52:25 PM
Last modification on : Thursday, November 21, 2019 - 1:38:51 AM

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Laribi Kamel, Denizon Nathalie, Besançon Anne, Farhi Jonathan, Lemaire Pierre, et al.. Blastic plasmacytoid dendritic cell neoplasm: From the origin of the cell to targeted therapies; Review. Biology of Blood and Marrow Transplantation, Elsevier, 2016, ⟨10.1016/j.bbmt.2016.03.022⟩. ⟨hal-01389961⟩

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