Treatment of children with refractory/relapse high risk langerhans cell histiocytosis with the combination of cytarabine, vindesine and prednisone.
Cytarabine
Langerhans cell histiocytosis
Refractory
Relapse
Risk organ
Journal
BMC pediatrics
ISSN: 1471-2431
Titre abrégé: BMC Pediatr
Pays: England
ID NLM: 100967804
Informations de publication
Date de publication:
03 Jan 2024
03 Jan 2024
Historique:
received:
07
05
2023
accepted:
05
12
2023
medline:
4
1
2024
pubmed:
4
1
2024
entrez:
3
1
2024
Statut:
epublish
Résumé
The patients with multisystem and risk organ involvement Langerhans cell histiocytosis (MS-RO + LCH) have poor prognosis. The patients with MS-LCH who failed front-line therapy have a high mortality rate and the standard salvage treatment has not been established. The combination of cytarabine (Ara-c), vincristine (VCR) and prednisone might be effective for refractory/relapse MS-RO + LCH, with low toxicity. We retrospectively analyzed pediatric refractory/relapse MS-RO + LCH patients treated with the low-dose Ara-c (100mg/m From January 2013 to December 2016, 13 patients receiving the low-dose Ara-c chemotherapy (LAC) and 7 patients receiving the high-dose Ara-c chemotherapy (HAC) were included in the study. 11 (84.6%) of the 13 patients treated with the LAC regimen and 6 (85.7%) of the 7 patients treated with the HAC regimen had response after four courses of the therapy. All patients in the study were alive during follow-up and the 3-year event-free survival rate (EFS) was 53.7% and 85.7% in the LAC and HAC groups. The most frequent adverse event was Grade 1/2 myelosuppression, which was observed in 38.5% (5/13) and 42.9% (3/7) of the patients receiving the LAC and HAC regimen. A combination of Ara-c, VDS and prednisone was effective and safe for some patients with refractory/relapse MS-RO + LCH. The high-dose Ara-c regimen was associated with a numerically higher EFS rate.
Sections du résumé
BACKGROUND
BACKGROUND
The patients with multisystem and risk organ involvement Langerhans cell histiocytosis (MS-RO + LCH) have poor prognosis. The patients with MS-LCH who failed front-line therapy have a high mortality rate and the standard salvage treatment has not been established. The combination of cytarabine (Ara-c), vincristine (VCR) and prednisone might be effective for refractory/relapse MS-RO + LCH, with low toxicity.
METHODS
METHODS
We retrospectively analyzed pediatric refractory/relapse MS-RO + LCH patients treated with the low-dose Ara-c (100mg/m
RESULTS
RESULTS
From January 2013 to December 2016, 13 patients receiving the low-dose Ara-c chemotherapy (LAC) and 7 patients receiving the high-dose Ara-c chemotherapy (HAC) were included in the study. 11 (84.6%) of the 13 patients treated with the LAC regimen and 6 (85.7%) of the 7 patients treated with the HAC regimen had response after four courses of the therapy. All patients in the study were alive during follow-up and the 3-year event-free survival rate (EFS) was 53.7% and 85.7% in the LAC and HAC groups. The most frequent adverse event was Grade 1/2 myelosuppression, which was observed in 38.5% (5/13) and 42.9% (3/7) of the patients receiving the LAC and HAC regimen.
CONCLUSIONS
CONCLUSIONS
A combination of Ara-c, VDS and prednisone was effective and safe for some patients with refractory/relapse MS-RO + LCH. The high-dose Ara-c regimen was associated with a numerically higher EFS rate.
Identifiants
pubmed: 38172736
doi: 10.1186/s12887-023-04465-5
pii: 10.1186/s12887-023-04465-5
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1Subventions
Organisme : the National Natural Science Foundation of China
ID : 82070202 and 82141119
Organisme : the National Natural Science Foundation of China
ID : 82070202 and 82141119
Organisme : the National Natural Science Foundation of China
ID : 82070202 and 82141119
Organisme : the National Natural Science Foundation of China
ID : 82070202 and 82141119
Organisme : the Beijing Natural Science Foundation
ID : 7232058
Organisme : the Beijing Natural Science Foundation
ID : 7232058
Organisme : the Beijing Natural Science Foundation
ID : 7232058
Organisme : the Beijing Natural Science Foundation
ID : 7232058
Organisme : the Capital's Funds for Health Improvement and Research
ID : 2020-2-2093, 2020-2-1141 and 2022-2-1141
Organisme : the Capital's Funds for Health Improvement and Research
ID : 2020-2-2093, 2020-2-1141 and 2022-2-1141
Organisme : the Capital's Funds for Health Improvement and Research
ID : 2020-2-2093, 2020-2-1141 and 2022-2-1141
Organisme : the Capital's Funds for Health Improvement and Research
ID : 2020-2-2093, 2020-2-1141 and 2022-2-1141
Organisme : the Special Fund of the Pediatric Medical Coordinated Development Center of Beijing Hospitals Authority
ID : XTZD20180201
Organisme : the Special Fund of the Pediatric Medical Coordinated Development Center of Beijing Hospitals Authority
ID : XTZD20180201
Organisme : the Special Fund of the Pediatric Medical Coordinated Development Center of Beijing Hospitals Authority
ID : XTZD20180201
Organisme : the Special Fund of the Pediatric Medical Coordinated Development Center of Beijing Hospitals Authority
ID : XTZD20180201
Informations de copyright
© 2023. The Author(s).
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