Journal
ONCOTARGET
Volume 6, Issue 22, Pages 19204-19216Publisher
IMPACT JOURNALS LLC
DOI: 10.18632/oncotarget.4146
Keywords
blastic plasmacytoid dendritic cell neoplasm; flow cytometry; maturation profile; acute leukemia; lymphoma
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Funding
- RETICS (Instituto de Salud Carlos III) [RD06/0020/0035, RD12/0036/0048]
- RETICS (Ministerio de Economia y Competitividad, Madrid, Spain) [RD06/0020/0035, RD12/0036/0048]
- RETICS (Fondos FEDER) [RD06/0020/0035, RD12/0036/0048]
- Junta de Castilla y Leon [EDU/878/2004]
- Fondo Social Europeo
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Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare subtype of leukemia/lymphoma, whose diagnosis can be difficult to achieve due to its clinical and biological heterogeneity, as well as its overlapping features with other hematologic malignancies. In this study we investigated whether the association between the maturational stage of tumor cells and the clinico-biological and prognostic features of the disease, based on the analysis of 46 BPDCN cases classified into three maturation-associated subgroups on immunophenotypic grounds. Our results show that blasts from cases with an immature plasmacytoid dendritic cell (pDC) phenotype exhibit an uncommon CD56(-) phenotype, coexisting with CD34(+) non-pDC tumor cells, typically in the absence of extramedullary (e.g. skin) disease at presentation. Conversely, patients with a more mature blast cell phenotype more frequently displayed skin/extramedullary involvement and spread into secondary lymphoid tissues. Despite the dismal outcome, acute lymphoblastic leukemia-type therapy (with central nervous system prophylaxis) and/or allogeneic stem cell transplantation appeared to be the only effective therapies. Overall, our findings indicate that the maturational profile of pDC blasts in BPDCN is highly heterogeneous and translates into a wide clinical spectrum -from acute leukemia to mature lymphoma-like behavior-, which may also lead to variable diagnosis and treatment.
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