title: Classification and clinical behavior of blastic plasmacytoid dendritic cell neoplasms according to their maturation-associated immunophenotypic profile.
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.
authors.affiliation: 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.
authors.forename: Lourdes
authors.initials: L
authors.lastname: Martín-Martín
authors.affiliation: 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.
authors.forename: Antonio
authors.initials: A
authors.lastname: López
authors.affiliation: Hematology Department and IBSAL, University Hospital of Salamanca, Salamanca, Spain.
authors.forename: Belén
authors.initials: B
authors.lastname: Vidriales
authors.affiliation: Hematology Department and IBSAL, University Hospital of Salamanca, Salamanca, Spain.
authors.forename: María Dolores
authors.initials: MD
authors.lastname: Caballero
authors.affiliation: Hematology Department, Hospital de Santo António dos Capuchos, Lisboa, Portugal.
authors.forename: António Silva
authors.initials: AS
authors.lastname: Rodrigues
authors.affiliation: Santa Luzia Medical Laboratory, Florianópolis, Santa Catarina, Brazil.
authors.forename: Silvia Inês
authors.initials: SI
authors.lastname: Ferreira
authors.affiliation: Clinical Hematology Department, Hospital de Santo António, Porto, Portugal.
authors.forename: Margarida
authors.initials: M
authors.lastname: Lima
authors.affiliation: Hematology Department, Hospital Universidade de Coimbra, Coimbra, Portugal.
authors.forename: Sérgio
authors.initials: S
authors.lastname: Almeida
authors.affiliation: Hematology Department, Hospital Nacional de Niños Dr. Carlos Sáenz Herrera, San José, Costa Rica.
authors.forename: Berta
authors.initials: B
authors.lastname: Valverde
authors.affiliation: Hematology Department, Hospital 12 de Octubre, Madrid, Spain.
authors.forename: Pilar
authors.initials: P
authors.lastname: Martínez
authors.affiliation: Pathology Department, Hospital del Mar, Barcelona. IMIM (Hospital del Mar Medical Research Institute), Barcelona, Spain.
authors.forename: Ana
authors.initials: A
authors.lastname: Ferrer
authors.affiliation: Immunology Department, Hospital São João, Porto, Portugal.
authors.forename: Jorge
authors.initials: J
authors.lastname: Candeias
authors.affiliation: Clinical Analysis and Immunology Department, Hospital Virgen de las Nieves, Granada, Spain.
authors.forename: Francisco
authors.initials: F
authors.lastname: Ruíz-Cabello
authors.affiliation: Hematology Department, Hospital General de Castellón, Castellón, Spain.
authors.forename: Josefa Marco
authors.initials: JM
authors.lastname: Buadesa
authors.affiliation: Hematology Department, University Hospital La Fé, Valencia, Spain.
authors.forename: Amparo
authors.initials: A
authors.lastname: Sempere
authors.affiliation: Hospital Clinic, Barcelona, Spain.
authors.forename: Neus
authors.initials: N
authors.lastname: Villamor
authors.affiliation: 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.
authors.forename: Alberto
authors.initials: A
authors.lastname: Orfao
authors.affiliation: 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.
authors.forename: Julia
authors.initials: J
authors.lastname: Almeida
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D003713:Dendritic Cells / Q000473:pathology*; D005260:Female; D005434:Flow Cytometry; D019337:Hematologic Neoplasms / Q000145:classification*; D006801:Humans; D016130:Immunophenotyping; D008297:Male; D008875:Middle Aged; D012878:Skin Neoplasms / Q000145:classification* / Q000473:pathology
keywords: acute leukemia; blastic plasmacytoid dendritic cell neoplasm; flow cytometry; lymphoma; maturation profile