Classification and clinical behavior of blastic plasmacytoid dendritic cell neoplasms according to their maturation-associated immunophenotypic profile
| العنوان: | Classification and clinical behavior of blastic plasmacytoid dendritic cell neoplasms according to their maturation-associated immunophenotypic profile |
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| المؤلفون: | Pilar Alejandra Saiz Martínez, Berta Valverde, Margarida Lima, Alberto Orfao, Josefa Marco Buadesa, Belén Vidriales, Silvia Inês Ferreira, Maria Dolores Caballero, António Silva Rodrigues, Jorge Candeias, Neus Villamor, Sérgio Almeida, Ana Ferrer, Francisco Ruiz-Cabello, Antonio López, Amparo Sempere, Julia Almeida, Lourdes Martín-Martín |
| المساهمون: | Junta de Castilla y León, European Commission, Ministerio de Economía y Competitividad (España), Instituto de Salud Carlos III, [Martín-Martín,L, López,A, Orfao,A, Almeida,J] Cancer Research Centre (IBMCC, USAL-CSIC), Institute for Biomedical Research of Salamanca (IBSAL) and Department of Medicine and Cytometry Service, University of Salamanca (USAL), Salamanca, Spain. [Vidriales,B, Caballero,MD] Hematology Department and IBSAL, University Hospital of Salamanca, Salamanca, Spain. [Silva Rodrigues,A] Hematology Department, Hospital de Santo António dos Capuchos, Lisboa, Portugal. [Ferreira,SI] Santa Luzia Medical Laboratory, Florianópolis, SC, Brazil. [Lima,M] Clinical Hematology Department, Hospital de Santo António, Porto, Portugal. [Almeida,S] Hematology Department, Hospital Universidade de Coimbra, Coimbra, Portugal. [Valverde,B] Hematology Department, Hospital Nacional de Niños Dr. Carlos Sáenz Herrera, San José, Costa Rica. [Martínez,P] Hematology Department, Hospital 12 de Octubre, Madrid, Spain. [Ferrer,A] Pathology Department, Hospital del Mar, Barcelona. IMIM (Hospital del Mar Medical Research Institute), Barcelona, Spain. [Candeias,J] Immunology Department, Hospital São João, Porto, Portugal. [Ruíz-Cabello,F] Clinical Analysis and Immunology Department, Hospital Virgen de las Nieves, Granada, Spain. [Marco Buadesa,J] Hematology Department, Hospital General de Castellón, Castellón, Spain. [Sempere,A] Hematology Department, University Hospital La Fé, Valencia, Spain. [Villamor,N] Hospital Clinic, Barcelona, Spain., This 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 Europeo. |
| المصدر: | Oncotarget r-FISABIO: Repositorio Institucional de Producción Científica Fundación para el Fomento de la Investigación Sanitaria y Biomédica de la Comunitat Valenciana (FISABIO) r-IIS La Fe. Repositorio Institucional de Producción Científica del Instituto de Investigación Sanitaria La Fe instname Scopus-Elsevier Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) Agência para a Sociedade do Conhecimento (UMIC)-FCT-Sociedade da Informação instacron:RCAAP Europe PubMed Central Digital.CSIC. Repositorio Institucional del CSIC r-FISABIO. Repositorio Institucional de Producción Científica |
| بيانات النشر: | IMPACT JOURNALS LLC, 2015. |
| سنة النشر: | 2015 |
| مصطلحات موضوعية: | Male, Pathology, Phenomena and Processes::Genetic Phenomena::Phenotype [Medical Subject Headings], Skin Neoplasms, Lymphoma, Diseases::Neoplasms::Neoplasms by Site::Hematologic Neoplasms [Medical Subject Headings], blastic plasmacytoid dendritic cell neoplasm, CD34, ComputingMilieux_LEGALASPECTSOFCOMPUTING, Plasmacytoid dendritic cell, Diseases::Neoplasms::Neoplasms by Histologic Type::Lymphoma [Medical Subject Headings], Plasmacitoma, Células Dendríticas, maturation profile, hemic and lymphatic diseases, Flow cytometry, acute leukemia, Citometría de Flujo, Child, Aged, 80 and over, Acute leukemia, Age Factors, Leucèmia, Middle Aged, Leucemia-Linfoma Linfoblástico de Células Precursoras, Diseases::Neoplasms::Neoplasms by Histologic Type::Neoplasms, Plasma Cell::Plasmacytoma [Medical Subject Headings], Leukemia, Linfoma, Oncology, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Surgical Procedures, Operative::Transplantation::Cell Transplantation::Stem Cell Transplantation [Medical Subject Headings], Analytical, Diagnostic and Therapeutic Techniques and Equipment::Diagnosis::Diagnostic Techniques and Procedures::Clinical Laboratory Techniques::Cytological Techniques::Cell Separation::Flow Cytometry [Medical Subject Headings], Hematologic Neoplasms, Female, Stem cell, Fenotipo, Research Paper, Adult, medicine.medical_specialty, Diseases::Neoplasms::Neoplasms by Histologic Type::Leukemia [Medical Subject Headings], Maturation profile, lymphoma, Biology, Immunophenotyping, Blastic plasmacytoid dendritic cell neoplasm, Precursor cell, Diseases::Neoplasms::Neoplasms by Histologic Type::Leukemia::Leukemia, Lymphoid::Precursor Cell Lymphoblastic Leukemia-Lymphoma [Medical Subject Headings], medicine, Humans, Leucemia, Limfomes -- Classificació, Aged, Anatomy::Cells::Antigen-Presenting Cells::Dendritic Cells [Medical Subject Headings], flow cytometry, Trasplante de Células Madre, Dendritic Cells, medicine.disease, Transplantation |
| الوصف: | This is an open-access article distributed under the terms of the Creative Commons Attribution License.-- et al. 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. This 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 Europeo. |
| وصف الملف: | application/pdf; application/vnd.oasis.opendocument.spreadsheet |
| تدمد: | 1949-2553 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9553e6939fbe79161d4c49f260964e43Test https://fundanet.fisabio.san.gva.es/publicaciones/ProdCientif/PublicacionFrw.aspx?id=10140Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....9553e6939fbe79161d4c49f260964e43 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 19492553 |
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