Classification and clinical behavior of blastic plasmacytoid dendritic cell neoplasms according to their maturation-associated immunophenotypic profile.

Martín-Martín, Lourdes; López, Antonio; Vidriales, Belén; Caballero, María Dolores; Silva Rodrigues, António; Ferreira, Silvia Inés; Lima, Margarida; Almeida, Sérgio; Valverde, Berta; Martínez, Pilar; Ferrer Del Alamo, Ana; Candeias, Jorge; Ruíz-Cabello, Francisco; Marco Buadesa, Josefa; Sempere, Amparo; Villamor, Neus; Orfao, Alberto; Almeida, Julia

Classification and clinical behavior of blastic plasmacytoid dendritic cell neoplasms according to their maturation-associated immunophenotypic profile.

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dc.contributor.author Martín-Martín, Lourdesca
dc.contributor.author López, Antonioca
dc.contributor.author Vidriales, Belénca
dc.contributor.author Caballero, María Doloresca
dc.contributor.author Silva Rodrigues, Antónioca
dc.contributor.author Ferreira, Silvia Inésca
dc.contributor.author Lima, Margaridaca
dc.contributor.author Almeida, Sérgioca
dc.contributor.author Valverde, Bertaca
dc.contributor.author Martínez, Pilarca
dc.contributor.author Ferrer Del Alamo, Anaca
dc.contributor.author Candeias, Jorgeca
dc.contributor.author Ruíz-Cabello, Franciscoca
dc.contributor.author Marco Buadesa, Josefaca
dc.contributor.author Sempere, Amparoca
dc.contributor.author Villamor, Neusca
dc.contributor.author Orfao, Albertoca
dc.contributor.author Almeida, Juliaca
dc.date.accessioned 2015-11-17T12:12:27Z
dc.date.available 2015-11-17T12:12:27Z
dc.date.issued 2015
dc.description.abstract 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.ca
dc.description.sponsorship This work was supported by grants RD06/0020/0035 and RD12/0036/0048 from RETICS (Instituto de Salud/nCarlos III, Ministerio de Economía y Competitividad, Madrid, Spain and Fondos FEDER) and grant EDU/878/2004 from Junta de Castilla y León and Fondo Social Europeo
dc.format.mimetype application/pdfca
dc.identifier.citation Martín-Martín L, López A, Vidriales B, Caballero MD, Rodrigues AS, Ferreira SI. et al. Classification and clinical behavior of blastic plasmacytoid dendritic cell neoplasms according to their maturation-associated immunophenotypic profile. Oncotarget. 2015 Aug 7;6(22):19204-16.ca
dc.identifier.doi http://dx.doi.org/10.18632/oncotarget.4146
dc.identifier.issn 1949-2553
dc.identifier.uri http://hdl.handle.net/10230/25111
dc.language.iso engca
dc.publisher Impact Journalsca
dc.relation.ispartof Oncotarget. 2015 Aug 7;6(22):19204-16
dc.rights.accessRights info:eu-repo/semantics/openAccessca
dc.rights.uri http://creativecommons.org/licenses/by/3.0/ca
dc.subject.other Leucèmiaca
dc.subject.other Limfomes -- Classificacióca
dc.title Classification and clinical behavior of blastic plasmacytoid dendritic cell neoplasms according to their maturation-associated immunophenotypic profile.ca
dc.type info:eu-repo/semantics/articleca
dc.type.version info:eu-repo/semantics/publishedVersionca

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