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Title: Classification and clinical behavior of blastic plasmacytoid dendritic cell neoplasms according to their maturation-associated immunophenotypic profile.
Authors: Martín-Martín, Lourdes
López, Antonio
Vidriales, Belén
Caballero, María Dolores
Silva Rodrigues, António
Ferreira, Silvia Inês
Lima, Margarida
Almeida, Sérgio
Valverde, Berta
Martínez, Pilar
Ferrer, Ana
Candeias, Jorge
Ruíz-Cabello, Francisco
Marco Buadesa, Josefa
Sempere, Amparo
Villamor, Neus
Orfao, Alberto
Almeida, Julia
metadata.dc.contributor.authoraffiliation: [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.
Keywords: Blastic plasmacytoid dendritic cell neoplasm;Flow cytometry;Maturation profile;Acute leukemia;Lymphoma;Células Dendríticas;Plasmacitoma;Leucemia;Linfoma;Trasplante de Células Madre;Citometría de Flujo;Fenotipo;Leucemia-Linfoma Linfoblástico de Células Precursoras
metadata.dc.subject.mesh: Medical Subject Headings::Anatomy::Cells::Antigen-Presenting Cells::Dendritic Cells
Medical Subject Headings::Diseases::Neoplasms::Neoplasms by Histologic Type::Neoplasms, Plasma Cell::Plasmacytoma
Medical Subject Headings::Diseases::Neoplasms::Neoplasms by Site::Hematologic Neoplasms
Medical Subject Headings::Diseases::Neoplasms::Neoplasms by Histologic Type::Leukemia
Medical Subject Headings::Diseases::Neoplasms::Neoplasms by Histologic Type::Lymphoma
Medical Subject Headings::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::Phenomena and Processes::Genetic Phenomena::Phenotype
Medical Subject Headings::Diseases::Neoplasms::Neoplasms by Histologic Type::Leukemia::Leukemia, Lymphoid::Precursor Cell Lymphoblastic Leukemia-Lymphoma
Issue Date: 25-May-2015
Publisher: Impact Journals
Citation: 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
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.
metadata.dc.identifier.doi: 10.18632/oncotarget.4146
ISSN: 1949-2553 (Online)
Appears in Collections:01- Artículos - Hospital Virgen de las Nieves

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