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香港唐氏综合征儿童急性白血病:回顾性研究

Acute Leukemia in Down Syndrome Children in Hong Kong: Retrospective Review.

作者信息

Lam Grace Kee See, Leung Alex Wing Kwan, Ha Shau Yin, Luk Chung Wing, Li Chak Ho, Ling Siu Cheung, Chiang Alan Kwok Shing, Li Chi Kong

机构信息

*Department of Pediatrics, Prince of Wales Hospital, the Chinese University of Hong Kong †Department of Pediatrics and Adolescent Medicine, Queen Mary Hospital, the University of Hong Kong ‡Department of Pediatrics, Queen Elizabeth Hospital §Department of Pediatrics and Adolescent Medicine, Tuen Mun Hospital ∥Department of Pediatrics & Adolescent Medicine, Princess Margaret Hospital, Hong Kong.

出版信息

J Pediatr Hematol Oncol. 2016 Mar;38(2):102-6. doi: 10.1097/MPH.0000000000000500.

DOI:10.1097/MPH.0000000000000500
PMID:26808367
Abstract

BACKGROUND

Children with Down syndrome (DS) are at higher risk of developing acute leukemia. Treatment continues to evolve as we accumulate better understanding of the distinctive clinical and biological features of acute leukemia in DS patients.

PROCEDURE

A retrospective review of the clinical features, treatment outcomes, and survival of DS children with acute leukemia in Hong Kong from 1993 to 2013 was conducted. Patients were identified from the registry of the Hong Kong Pediatric Hematology and Oncology study group.

RESULTS

This cohort included a total of 29 patients with DS. Ten were diagnosed with acute lymphoblastic leukemia and 19 had acute myeloid leukemia (AML). The mean follow-up duration was 8.3 years (range, 0.6 mo to 18.1 y). The 5-year overall survival and event-free survival for DS-acute lymphoblastic leukemia and DS-AML were 65.6%, 54.9%, 89.5%, and 89.5%, respectively.

CONCLUSIONS

The clinical characteristics and treatment outcomes of DS patients with acute leukemia in Hong Kong were comparable with results from other international study groups. Patients with DS-AML had a better prognosis.

摘要

背景

唐氏综合征(DS)患儿患急性白血病的风险更高。随着我们对DS患者急性白血病独特的临床和生物学特征有了更深入的了解,治疗方法也在不断发展。

程序

对1993年至2013年香港地区患有急性白血病的DS患儿的临床特征、治疗结果和生存率进行了回顾性研究。患者从香港儿科血液学和肿瘤学研究组的登记处中确定。

结果

该队列共有29例DS患者。10例被诊断为急性淋巴细胞白血病,19例患有急性髓系白血病(AML)。平均随访时间为8.3年(范围为0.6个月至18.1年)。DS急性淋巴细胞白血病和DS-AML的5年总生存率和无事件生存率分别为65.6%、54.9%、89.5%和89.5%。

结论

香港地区DS急性白血病患者的临床特征和治疗结果与其他国际研究组的结果相当。DS-AML患者的预后较好。

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