Classification and clinical behavior of blastic plasmacytoid dendritic cell neoplasms according to their maturation-associated immunophenotypic profile
Por:
Martin-Martin, L, Lopez, A, Vidriales, B, Caballero, M, Rodrigues, A, Ferreira, S, Lima, M, Almeida, S, Valverde, B, Martinez, P, Ferrer, A, Candeias, J, Ruiz-Cabello, F, Buadesa, J, Sempere, A, Villamor, N, Orfao, A and Almeida, J
Publicada:
7 ago 2015
Categoría:
Oncology
Resumen:
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.
Filiaciones:
Martin-Martin, L:
Univ Salamanca USAL, Inst Biomed Res Salamanca IBSAL, Canc Res Ctr IBMCC USAL CSIC, Salamanca, Spain
Univ Salamanca USAL, Dept Med & Cytometry Serv, Salamanca, Spain
Lopez, A:
Univ Salamanca USAL, Inst Biomed Res Salamanca IBSAL, Canc Res Ctr IBMCC USAL CSIC, Salamanca, Spain
Univ Salamanca USAL, Dept Med & Cytometry Serv, Salamanca, Spain
Vidriales, B:
Univ Hosp Salamanca, Dept Hematol, Salamanca, Spain
Univ Hosp Salamanca, IBSAL, Salamanca, Spain
Caballero, M:
Univ Hosp Salamanca, Dept Hematol, Salamanca, Spain
Univ Hosp Salamanca, IBSAL, Salamanca, Spain
Rodrigues, A:
Hosp Santo Antonio Capuchos, Dept Hematol, Lisbon, Portugal
Ferreira, S:
Santa Luzia Med Lab, Florianopolis, SC, Brazil
Lima, M:
Hosp Santo Antonio, Clin Hematol Dept, Oporto, Portugal
Almeida, S:
Hosp Univ Coimbra, Dept Hematol, Coimbra, Portugal
Valverde, B:
Hosp Nacl Ninos Dr Carlos Saenz Herrera, Dept Hematol, San Jose, Costa Rica
Martinez, P:
Hosp 12 Octubre, Dept Hematol, E-28041 Madrid, Spain
Ferrer, A:
Hosp Mar, Dept Pathol, Barcelona, Spain
IMIM Hosp Mar Med Res Inst, Barcelona, Spain
Candeias, J:
Hosp Sao Joao, Dept Immunol, Oporto, Portugal
Ruiz-Cabello, F:
Hosp Virgen Ias Nieves, Clin Anal & Immunol Dept, Granada, Spain
:
Hosp Gen Castellon, Dept Hematol, Castellon de La Plana, Spain
Sempere, A:
Univ Hosp La Fe, Dept Hematol, Valencia, Spain
Villamor, N:
Hosp Clin Barcelona, Barcelona, Spain
Orfao, A:
Univ Salamanca USAL, Inst Biomed Res Salamanca IBSAL, Canc Res Ctr IBMCC USAL CSIC, Salamanca, Spain
Univ Salamanca USAL, Dept Med & Cytometry Serv, Salamanca, Spain
Almeida, J:
Univ Salamanca USAL, Inst Biomed Res Salamanca IBSAL, Canc Res Ctr IBMCC USAL CSIC, Salamanca, Spain
Univ Salamanca USAL, Dept Med & Cytometry Serv, Salamanca, Spain
Hybrid Gold, Green Published
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