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发展中国家儿童伯基特淋巴瘤的临床病程及预后因素:巴西单中心经验

Clinical course and prognostic factors of children with Burkitt's lymphoma in a developing country: the experience of a single centre in Brazil.

作者信息

Cunha Keyla Christy Christine Mendes Sampaio, Oliveira Maria Christina Lopes Araujo, Gomes Ana Cecília Silva, de Castro Lucia Porto Fonseca, Viana Marcos Borato

机构信息

Universidade Federal de Minas Gerais - UFMG, Belo Horizonte, MG, Brazil.

出版信息

Rev Bras Hematol Hemoter. 2012;34(5):361-6. doi: 10.5581/1516-8484.20120093.

Abstract

OBJECTIVE

Burkitt's lymphoma is the most common subtype of non-Hodgkin lymphoma in children. The aim of this study was to characterize the clinical course and prognostic factors of children and adolescents with Burkitt's lymphoma treated in the Hematology Unit of Hospital das Clínicas, Universidade Federal de Minas Gerais (UFMG).

METHODS

A retrospective cohort study was made of 50 consecutive cases of children and adolescents aged 16 years or less with Burkitt's lymphoma admitted between January 1981 and December 2007. Prognostic factors associated with death were evaluated using the Kaplan-Meier method and compared by the two-tailed log-rank test.

RESULTS

The median age at diagnosis was 4.7 years. Most patients had abdominal tumors (66.7%) and advanced disease (68.9%) at diagnosis. Thirty-eight patients (84.4%) achieved complete clinical remission and 33 (73.3%) were alive at the first remission. Twelve children (26.7%) died. The median follow-up was 35 months with the probability of overall survival being 73% (89.2% and 35.7% for patients with uric acid < 7 mg/dL and ≥ 7.0 mg/dL, respectively - p-value < 0.001). Uric acid was the only significant prognostic factor at diagnosis.

CONCLUSION

Our findings confirm the favorable prognosis of children with Burkitt's lymphoma even when treated with intermediate doses of methotrexate (500 mg/m2). Survival was significantly lower for individuals with concentrations of uric acid > 7 mg/dL.

摘要

目的

伯基特淋巴瘤是儿童非霍奇金淋巴瘤最常见的亚型。本研究的目的是描述在米纳斯吉拉斯联邦大学临床医院血液科接受治疗的儿童和青少年伯基特淋巴瘤的临床病程及预后因素。

方法

对1981年1月至2007年12月期间收治的50例16岁及以下儿童和青少年伯基特淋巴瘤连续病例进行回顾性队列研究。采用Kaplan-Meier法评估与死亡相关的预后因素,并通过双侧对数秩检验进行比较。

结果

诊断时的中位年龄为4.7岁。大多数患者在诊断时有腹部肿瘤(66.7%)和晚期疾病(68.9%)。38例患者(84.4%)实现了完全临床缓解,33例(73.3%)在首次缓解时存活。12名儿童(26.7%)死亡。中位随访时间为35个月,总生存率为73%(尿酸<7mg/dL和≥7.0mg/dL的患者分别为89.2%和35.7% - p值<0.001)。尿酸是诊断时唯一显著的预后因素。

结论

我们的研究结果证实,即使使用中等剂量的甲氨蝶呤(500mg/m²)治疗,儿童伯基特淋巴瘤的预后也良好。尿酸浓度>7mg/dL的个体生存率显著较低。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5fbe/3486827/57913f96ff36/rbhh-34-361-g01.jpg

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