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。