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儿童和青少年与治疗相关的髓系肿瘤

Therapy-related myeloid neoplasms in children and adolescents.

作者信息

Cho Hee Won, Choi Young Bae, Yi Eun Sang, Lee Ji Won, Sung Ki Woong, Koo Hong Hoe, Yoo Keon Hee

机构信息

Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

Department of Pediatrics, Chung-Ang University Hospital, Seoul, Korea.

出版信息

Blood Res. 2016 Dec;51(4):242-248. doi: 10.5045/br.2016.51.4.242. Epub 2016 Dec 23.

DOI:10.5045/br.2016.51.4.242
PMID:28090486
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5234240/
Abstract

BACKGROUND

This retrospective study aimed to characterize and analyze the outcome of therapy-related myeloid neoplasms (t-MNs) in children and adolescents.

METHODS

The medical records of 16 patients under 21 years of age at the time of t-MN diagnosis were reviewed.

RESULTS

The median patient age was 11.5 years (range, 1.6-20.4 yr). Twelve patients had therapy-related acute myeloid leukemia, 3 patients had myelodysplastic syndrome, and 1 patient had chronic myelomonocytic leukemia. The median latency period was 29 months (range, 11-68 mo). Fourteen patients had cytogenetic aberrations, 8 of whom had an 11q23 abnormality. Of the 13 patients treated with curative intent, 12 patients received myeloid-type induction therapy that led to complete remission (CR) in 8 patients. Nine patients underwent allogeneic transplantation; 4 patients did not undergo transplantation due to chemotherapy-related toxic death (N=3) or parental refusal (N=1). The 5-year overall survival and event-free survival of the 13 patients treated with a curative intent were 46.2% and 30.8%, respectively. For the 9 patients who underwent allogeneic transplantation, the 5-year event-free survival was 66.7%.

CONCLUSION

A significant proportion of young patients with t-MNs can experience long-term survival, and allogeneic transplantation plays a key role for attaining cure in these patients.

摘要

背景

这项回顾性研究旨在描述和分析儿童及青少年治疗相关髓系肿瘤(t-MNs)的治疗结果。

方法

回顾了16例在t-MN诊断时年龄小于21岁患者的病历。

结果

患者中位年龄为11.5岁(范围1.6 - 20.4岁)。12例患者患有治疗相关急性髓系白血病,3例患者患有骨髓增生异常综合征,1例患者患有慢性粒单核细胞白血病。中位潜伏期为29个月(范围11 - 68个月)。14例患者有细胞遗传学异常,其中8例有11q23异常。在13例接受根治性治疗的患者中,12例接受了髓系诱导治疗,8例患者达到完全缓解(CR)。9例患者接受了异基因移植;4例患者因化疗相关毒性死亡(n = 3)或家长拒绝(n = 1)未接受移植。13例接受根治性治疗患者的5年总生存率和无事件生存率分别为46.2%和30.8%。对于9例接受异基因移植的患者,5年无事件生存率为6,6.7%。

结论

相当一部分年轻的t-MNs患者可实现长期生存,异基因移植对这些患者的治愈起着关键作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/db0f/5234240/c8ac43b5da19/br-51-242-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/db0f/5234240/d164925a7485/br-51-242-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/db0f/5234240/4d448c36288b/br-51-242-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/db0f/5234240/c8ac43b5da19/br-51-242-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/db0f/5234240/d164925a7485/br-51-242-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/db0f/5234240/4d448c36288b/br-51-242-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/db0f/5234240/c8ac43b5da19/br-51-242-g003.jpg

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