RT Journal Article
T1 Classification and clinical behavior of blastic plasmacytoid dendritic cell neoplasms according to their maturation-associated immunophenotypic profile.
A1 Martín-Martín, Lourdes
A1 López, Antonio
A1 Vidriales, Belén
A1 Caballero, María Dolores
A1 Silva Rodrigues, António
A1 Ferreira, Silvia Inês
A1 Lima, Margarida
A1 Almeida, Sérgio
A1 Valverde, Berta
A1 Martínez, Pilar
A1 Ferrer, Ana
A1 Candeias, Jorge
A1 Ruíz-Cabello, Francisco
A1 Marco Buadesa, Josefa
A1 Sempere, Amparo
A1 Villamor, Neus
A1 Orfao, Alberto
A1 Almeida, Julia
K1 Blastic plasmacytoid dendritic cell neoplasm
K1 Flow cytometry
K1 Maturation profile
K1 Acute leukemia
K1 Lymphoma
K1 Células Dendríticas
K1 Plasmacitoma
K1 Leucemia
K1 Linfoma
K1 Trasplante de Células Madre
K1 Citometría de Flujo
K1 Fenotipo
K1 Leucemia-Linfoma Linfoblástico de Células Precursoras
AB 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.
PB Impact Journals
YR 2015
FD 2015-05-25
LK http://hdl.handle.net/10668/2077
UL http://hdl.handle.net/10668/2077
LA en
NO Martín-Martín, L., López, A., Vidriales, B., Dolores Caballero, M., Silva Rodrigues, A., Inês Ferreira, S., Lima, M., Almeida, S., Valverde, B., Martínez, P., Ferrer, A., Candeias, J., Ruiz-Cabello, F., Marco Buadesa, J., Sempere, A., Villamor, N., Orfao, A.,  Almeida, J.. Classification and clinical behavior of blastic plasmacytoid dendritic cell neoplasms according to their maturation-associated immunophenotypic profile. Oncotarget. 2015;  6(22), 19204-19216
NO JOURNAL ARTICLE;
DS RISalud
RD Jun 1, 2025