Division of Cellular and Molecular Pathology, Department of Pathology, University of Cambridge, Cambridge, UK.
Department of Life Sciences, Birmingham City University, Birmingham, UK.
Br J Haematol. 2021 Jan;192(2):354-365. doi: 10.1111/bjh.17043. Epub 2020 Sep 3.
Burkitt lymphoma (BL) accounts for almost two-thirds of all B-cell non-Hodgkin lymphoma (B-NHL) in children and adolescents and is characterised by a MYC translocation and rapid cell turnover. Intensive chemotherapeutic regimens have been developed in recent decades, including the lymphomes malins B (LMB) protocol, which have resulted in a survival rate in excess of 90%. Recent clinical trials have focused on immunochemotherapy, with the addition of rituximab to chemotherapeutic backbones, showing encouraging results. Despite these advances, relapse and refractory disease occurs in up to 10% of patients and salvage options for these carry a dismal prognosis. Efforts to better understand the molecular and functional characteristics driving relapse and refractory disease may help improve this prognosis. This study has established a paediatric BL patient-derived xenograft (PDX) resource which captures and maintains tumour heterogeneity, may be used to better characterise tumours and identify cell populations responsible for therapy resistance.
伯基特淋巴瘤（BL）占儿童和青少年所有 B 细胞非霍奇金淋巴瘤（B-NHL）的近三分之二，其特征是 MYC 易位和快速细胞更新。近几十年来已经开发了强化化疗方案，包括淋巴母细胞瘤（LMB）方案，其生存率超过 90%。最近的临床试验侧重于免疫化疗，在化疗骨干中加入利妥昔单抗，取得了令人鼓舞的结果。尽管取得了这些进展，但仍有多达 10%的患者出现复发和难治性疾病，这些患者的挽救治疗选择预后较差。为了更好地了解导致复发和难治性疾病的分子和功能特征，可能有助于改善这一预后。本研究建立了一个儿科 BL 患者来源异种移植（PDX）资源，该资源可捕获和维持肿瘤异质性，可用于更好地描述肿瘤并确定对治疗耐药的细胞群体。
