Please use this identifier to cite or link to this item: http://hdl.handle.net/2445/178701
Title: Blastic plasmacytoid dendritic cell neoplasm frequently shows occult central nervous system involvement at diagnosis and benefits from intrathecal therapy
Author: Martin Martin, Lourdes
Almeida, Julia
Pomares, Hector
González Barca, Eva
Bravo, Pilar
Giménez, Teresa
Heras, Cecilia
Queizán, José-Antonio
Pérez-Ceballos, Elena
Martínez, Violeta
Alonso, Natalia
Calvo, Carlota
Álvarez, Rodolfo
Caballero, María Dolores
Orfao, Alberto
Keywords: Cèl·lules dendrítiques
Sistema nerviós central
Citometria de fluxe
Dendritic cells
Central nervous system
Flow cytometry
Issue Date: 1-Mar-2016
Publisher: Impact Journals
Abstract: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare aggressive myeloid neoplasm which shows a high rate of central nervous system (CNS) recurrence and overall survival (OS) of <1 year. Despite this, screening for CNS involvement is not routinely performed at diagnosis and intrathecal (IT) prophylaxis is not regularly administered in BPDCN. Here, we prospectively evaluated 13 consecutive BPDCN patients for the presence of CNS involvement by flow cytometry. Despite none of the patients presented with neurological symptoms, occult CNS involvement was detected in 6/10 cases evaluated at diagnosis and 3/3 studied at relapse/progression. BPDCN patients evaluated at diagnosis received IT treatment -either CNS prophylaxis (n = 4) or active therapy (n = 6)- and all but one remain alive (median follow-up of 20 months). In contrast, all three patients assessed at relapse/progression died. The potential benefit of IT treatment administered early at diagnosis on OS and CNS recurrence-free survival of BPDCN was further confirmed in a retrospective cohort of another 23 BPDCN patients. Our results show that BPDCN patients studied at diagnosis frequently display occult CNS involvement; moreover, they also indicate that treatment of occult CNS disease might lead to a dramatically improved outcome of BPDCN.
Note: Reproducció del document publicat a: https://doi.org/10.18632/oncotarget.7101
It is part of: Oncotarget, 2016, vol. 7, num. 9, p. 10174-10181
URI: http://hdl.handle.net/2445/178701
Related resource: https://doi.org/10.18632/oncotarget.7101
ISSN: 1949-2553
Appears in Collections:Articles publicats en revistes (Ciències Clíniques)

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