2024-03-29T15:53:32Zhttp://digital.csic.es/dspace-oai/requestoai:digital.csic.es:10261/1684352020-12-13T09:14:03Zcom_10261_112com_10261_1col_10261_365
Martín-Martín, Lourdes
Almeida, Julia
Pomares, Helena
González-Barca, Eva
Bravo, Pilar
Giménez, Teresa
Heras, Cecilia
Queizán, José-Antonio
Pérez-Ceballos, Elena
Martínez, Violeta
Alonso, Natalia
Calvo, Carlota
Álvarez, Rodolfo
Caballero, Maria Dolores
Orfao, Alberto
2018-08-08T09:25:06Z
2018-08-08T09:25:06Z
2016
Oncotarget 7(9): 10174-10181 (2016)
http://hdl.handle.net/10261/168435
10.18632/oncotarget.7101
http://dx.doi.org/10.13039/501100000780
http://dx.doi.org/10.13039/501100004587
http://dx.doi.org/10.13039/501100003329
http://dx.doi.org/10.13039/501100014180
26840087
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.
eng
http://creativecommons.org/licenses/by/3.0/
openAccess
Flow cytometry
ALL therapy
Intrathecal prophylaxis
Blastic plasmacytoid dendritic cell neoplasm
Central nervous system
Blastic plasmacytoid dendritic cell neoplasm frequently shows occult central nervous system involvement at diagnosis and benefits from intrathecal therapy
artículo