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2016 ; 7
(9
): 10174-81
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Blastic plasmacytoid dendritic cell neoplasm frequently shows occult central
nervous system involvement at diagnosis and benefits from intrathecal therapy
#MMPMID26840087
Martín-Martín L
; Almeida J
; Pomares H
; González-Barca E
; Bravo P
; Giménez T
; Heras C
; Queizán JA
; Pérez-Ceballos E
; Martínez V
; Alonso N
; Calvo C
; Álvarez R
; Caballero MD
; Orfao A
Oncotarget
2016[Mar]; 7
(9
): 10174-81
PMID26840087
show ga
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.
|Adolescent
[MESH]
|Adult
[MESH]
|Aged
[MESH]
|Central Nervous System Neoplasms/*secondary/*therapy
[MESH]