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

2015; Impact Journals LLC; Volume: 6; Issue: 22 Linguagem: Inglês

10.18632/oncotarget.4146

1949-2553

Lourdes Martín‐Martín, Antonio López, María‐Belén Vidriales, Marı́a Dolores Caballero, António Silva Rodrigues, Silvia Inês Alejandra Cordoba Pires Ferreira, Margarida Lima, Sérgio Monteiro de Almeida, Berta Valverde, Pilar Martı́nez, Ana Ferrer, Jorge Candeias, Francisco Ruiz‐Cabello, Josefa Marco Buadesa, Amparo Sempere, Neus Villamor, Alberto Órfão, Júlia Almeida,

Lymphoma Diagnosis and Treatment

// Lourdes Martín-Martín 1 , Antonio López 1 , Belén Vidriales 2 , María Dolores Caballero 2 , António Silva Rodrigues 3 , Silvia Inês Ferreira 4 , Margarida Lima 5 , Sérgio Almeida 6 , Berta Valverde 7 , Pilar Martínez 8 , Ana Ferrer 9 , Jorge Candeias 10 , Francisco Ruíz-Cabello 11 , Josefa Marco Buadesa 12 , Amparo Sempere 13 , Neus Villamor 14 , Alberto Orfao 1, * , Julia Almeida 1, * 1 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 2 Hematology Department and IBSAL, University Hospital of Salamanca, Salamanca, Spain 3 Hematology Department, Hospital de Santo António dos Capuchos, Lisboa, Portugal 4 Santa Luzia Medical Laboratory, Florianópolis, SC, Brazil 5 Clinical Hematology Department, Hospital de Santo António, Porto, Portugal 6 Hematology Department, Hospital Universidade de Coimbra, Coimbra, Portugal 7 Hematology Department, Hospital Nacional de Niños Dr. Carlos Sáenz Herrera, San José, Costa Rica 8 Hematology Department, Hospital 12 de Octubre, Madrid, Spain 9 Pathology Department, Hospital del Mar, Barcelona. IMIM (Hospital del Mar Medical Research Institute), Barcelona, Spain 10 Immunology Department, Hospital São João, Porto, Portugal 11 Clinical Analysis and Immunology Department, Hospital Virgen de las Nieves, Granada, Spain 12 Hematology Department, Hospital General de Castellón, Castellón, Spain 13 Hematology Department, University Hospital La Fé, Valencia, Spain 14 Hospital Clinic, Barcelona, Spain * These authors have contributed equally to this work Correspondence to: Alberto Orfao, e-mail: orfao@usal.es 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 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.