期刊
ONCOTARGET
卷 7, 期 9, 页码 10174-10181出版社
IMPACT JOURNALS LLC
DOI: 10.18632/oncotarget.7101
关键词
blastic plasmacytoid dendritic cell neoplasm; central nervous system; intrathecal prophylaxis; ALL therapy; flow cytometry
资金
- RETICS (Instituto de Salud Carlos III, Ministerio de Economia y Competitividad, Madrid, Spain) [RD06/0020/0035, RD12/0036/0048]
- RETICS (Fondos FEDER) [RD06/0020/0035, RD12/0036/0048]
- Junta de Castilla y Leon [EDU/878/2004]
- Fondo Social Europeo
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.
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