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儿童及青少年伯基特淋巴瘤/白血病

Burkitt Lymphoma/Leukaemia in Children & Young Adolescents.

作者信息

O'Rourke E, Malone A, O'Marcaigh A, Storey L, Betts D, McDermott M, Smith O P

机构信息

Department of Haematology, Our Lady's Children's Hospital Crumlin.

UCD School of Medicine, University College Dublin.

出版信息

Ir Med J. 2020 Jan 16;113(1):6.

PMID:32298568
Abstract

Aims Burkitt Lymphoma (BL) accounts for approximately 40% of childhood non-Hodgkin Lymphoma (NHL) in the developed world. Survival rates have improved dramatically in recent years, a success attributed to better use of poly-chemotherapy and targeted immunotherapy. Nevertheless, relapse is unpredictable and carries a dismal prognosis. We report on event-free survival (EFS) and overall survival (OS) rates in the Republic of Ireland (ROI) during 2000-2017, and evaluate novel predictors of outcome. Methods Data was collected by retrospective review of patient medical records. Results Thirty-three patients were identified (twenty-five [76%] males, eight [24%] females), fourteen [42%] having stage III disease at presentation. Six [18%] had stage IV disease. Five [15%] had refractory disease; one salvaged with allogeneic stem cell transplantation. Of the four [12%] who died; two [50%] had weights >99th centile, one [25%] >90th centile. One died during induction from refractory lactic acidosis, one from early relapse. Discussion EFS and OS was 85% and 89% respectively; in keeping with the best international standards. Obesity appears to be a poor predictor of outcome in our cohort.

摘要

目的

在发达国家,伯基特淋巴瘤(BL)约占儿童非霍奇金淋巴瘤(NHL)的40%。近年来生存率有了显著提高,这一成功归因于多化疗和靶向免疫疗法的更好应用。然而,复发是不可预测的,预后不佳。我们报告了2000年至2017年爱尔兰共和国(ROI)的无事件生存率(EFS)和总生存率(OS),并评估了新的预后预测因素。方法:通过回顾性审查患者病历收集数据。结果:共确定33例患者(25例[76%]为男性,8例[24%]为女性),14例[42%]初诊时为III期疾病。6例[18%]为IV期疾病。5例[15%]为难治性疾病;1例通过异基因干细胞移植挽救。4例[12%]死亡患者中;2例[50%]体重>第99百分位数,1例[25%]>第90百分位数。1例在诱导期死于难治性乳酸酸中毒,1例死于早期复发。讨论:EFS和OS分别为85%和89%;与国际最佳标准一致。在我们的队列中,肥胖似乎是一个不良的预后预测因素。

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