Deprecated: Implicit conversion from float 213.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 213.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 213.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 213.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 213.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 213.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 213.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 247.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 247.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 247.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 247.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\28870016
.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Korean+J+Intern+Med
2017 ; 32
(5
): 890-899
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Clinical features and treatment outcomes of blastic plasmacytoid dendritic cell
neoplasm: a single-center experience in Korea
#MMPMID28870016
Kim HS
; Kim HJ
; Kim SH
; Choi JY
; Ko YH
; Kim WS
; Jung CW
; Kim SJ
Korean J Intern Med
2017[Sep]; 32
(5
): 890-899
PMID28870016
show ga
BACKGROUND/AIMS: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare
hematologic malignancy that typically presents in the form of skin manifestations
with or without lymph node and bone marrow involvement. Given its rarity and
recent recognition as a distinct pathological entity, no standard of treatment
exists for this aggressive disease and its prognosis is particularly dismal.
METHODS: We retrospectively analyzed clinical features and treatment outcomes of
patients who were diagnosed with BPDCN between 2000 and 2014. RESULTS: Ten
patients had a median age at diagnosis of 41 years (range, 18 to 79), and seven
patients were male. Sites of disease involvement were the skin (n = 7), lymph
node (n = 5), bone marrow (n = 2), liver (n = 2), spleen (n = 2), and soft tissue
(n = 1). Intensified chemotherapy regimens such as hyperCVAD regimen
(cyclophosphamide, vincristine, doxorubicin, dexamethasone, methotrexate,
cytarabine), and VPDL (vincristine, methylprednisolone, daunorubicin,
L-asparaginase) were used as a first-line treatment. Although all patients
treated with intensified chemotherapy showed an objective response (five patients
with complete response) with median progression-free survival of 11.2 months
(range 6.2 to 19.4), complete remission was not sustained for more than 2 years
in any case. The response was relatively long-lived compared with previously
reported CHOP (doxorubicin, cyclophosphamide, vincristine, prednisone)-like
regimens, but the above regimens do not result in long-term remission.
CONCLUSIONS: All patients treated with hyperCVAD or VPDL showed an objective
response, but the duration of response was relatively short. Thus, the
development of more effective induction as well as consolidation treatment
strategy should be warranted to improve this rare disease entity.
|Adolescent
[MESH]
|Adult
[MESH]
|Aged
[MESH]
|Antineoplastic Combined Chemotherapy Protocols/adverse effects/*therapeutic use
[MESH]