Classification and clinical behavior of blastic plasmacytoid dendritic cell neoplasms according to their maturation-associated immunophenotypic profile
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Lourdes Martín-Martín1, Antonio López1, Belén Vidriales2, María Dolores Caballero2, António Silva Rodrigues3, Silvia Inês Ferreira4, Margarida Lima5, Sérgio Almeida6, Berta Valverde7, Pilar Martínez8, Ana Ferrer9, Jorge Candeias10, Francisco Ruíz-Cabello11, Josefa Marco Buadesa12, Amparo Sempere13, Neus Villamor14, Alberto Orfao1,*, Julia Almeida1,*
1Cancer 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
2Hematology Department and IBSAL, University Hospital of Salamanca, Salamanca, Spain
3Hematology Department, Hospital de Santo António dos Capuchos, Lisboa, Portugal
4Santa Luzia Medical Laboratory, Florianópolis, SC, Brazil
5Clinical Hematology Department, Hospital de Santo António, Porto, Portugal
6Hematology Department, Hospital Universidade de Coimbra, Coimbra, Portugal
7Hematology Department, Hospital Nacional de Niños Dr. Carlos Sáenz Herrera, San José, Costa Rica
8Hematology Department, Hospital 12 de Octubre, Madrid, Spain
9Pathology Department, Hospital del Mar, Barcelona. IMIM (Hospital del Mar Medical Research Institute), Barcelona, Spain
10Immunology Department, Hospital São João, Porto, Portugal
11Clinical Analysis and Immunology Department, Hospital Virgen de las Nieves, Granada, Spain
12Hematology Department, Hospital General de Castellón, Castellón, Spain
13Hematology Department, University Hospital La Fé, Valencia, Spain
14Hospital Clinic, Barcelona, Spain
*These authors have contributed equally to this work
Alberto Orfao, e-mail: [email protected]
Keywords: blastic plasmacytoid dendritic cell neoplasm, flow cytometry, maturation profile, acute leukemia, lymphoma
Received: April 15, 2015 Accepted: May 11, 2015 Published: May 25, 2015
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.
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