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

dc.contributor.authorMartín-Martín, L.
dc.contributor.authorLópez, A.
dc.contributor.authorVidriales, B.
dc.contributor.authorCaballero, M.
dc.contributor.authorRodrigues, A.
dc.contributor.authorFerreira, S.
dc.contributor.authorLima, M.
dc.contributor.authorAlmeida, S.
dc.contributor.authorValverde, B.
dc.contributor.authorMartínez, P.
dc.contributor.authorFerrer, A.
dc.contributor.authorCandeias, J.
dc.contributor.authorRuíz-Cabello, F.
dc.contributor.authorBuadesa, J.
dc.contributor.authorSempere, A.
dc.contributor.authorVillamor, N.
dc.contributor.authorOrfao, A.
dc.contributor.authorAlmeida, J.
dc.date.accessioned2016-07-26T10:04:12Z
dc.date.available2016-07-26T10:04:12Z
dc.date.issued2015-08-07
dc.description.abstractBlastic 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.pt_PT
dc.description.sponsorshipThis work was supported by grants RD06/0020/0035 and RD12/0036/0048 from RETICS (Instituto de Salud Carlos 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 Europeopt_PT
dc.identifier.citationOncotarget. 2015 Aug 7;6(22):19204-16pt_PT
dc.identifier.doi10.18632/oncotarget.4146pt_PT
dc.identifier.issn1949-2553
dc.identifier.urihttp://hdl.handle.net/10400.16/1967
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherImpact Journalspt_PT
dc.relation.publisherversionhttp://www.impactjournals.com/oncotarget/index.php?journal=oncotarget&page=article&op=view&path[]=4146&pubmed-linkout=1pt_PT
dc.subjectblastic plasmacytoid dendritic cell neoplasmpt_PT
dc.subjectflow cytometrypt_PT
dc.subjectmaturation profilept_PT
dc.subjectacute leukemiapt_PT
dc.titleClassification and clinical behavior of blastic plasmacytoid dendritic cell neoplasms according to their maturation-associated immunophenotypic profilept_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.conferencePlaceUnited States of Americapt_PT
oaire.citation.endPage19216pt_PT
oaire.citation.issue22pt_PT
oaire.citation.startPage19204pt_PT
oaire.citation.titleOncotargetpt_PT
oaire.citation.volume6pt_PT
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT

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