Casulo Carla, Friedberg Jonathan W
James P. Wilmot Cancer Institute, University of Rochester Medical Center, 601 Elmwood Avenue Box 704, Rochester, NY, 14642, USA.
James P. Wilmot Cancer Institute, University of Rochester Medical Center, 601 Elmwood Avenue Box 704, Rochester, NY, 14642, USA.
Best Pract Res Clin Haematol. 2018 Sep;31(3):279-284. doi: 10.1016/j.beha.2018.07.013. Epub 2018 Jul 23.
Burkitt lymphoma (BL) is a rare, aggressive subtype of non-Hodgkin lymphoma affecting approximately 1500 patients per year. Three forms of BL exist (sporadic, endemic, immunodeficiency associated) and the endemic form was first discovered as being driven by the Epstein Barr virus in areas of the world where malaria is prevalent. BL has the characteristic t8; 14 cytogenetic translocation that leads to constitutive activation of the MYC gene, which drives BL cell division. Therapy of BL has resulted in cure for many patients but significant toxicity and treatment related complications remains problematic in the approach to BL therapy. Treatment options for relapsed and refractory disease remain limited however novel treatments are being studied to block MYC activation, and cold lead to promising options for patients with relapsed and refractory disease.
伯基特淋巴瘤(BL)是一种罕见的侵袭性非霍奇金淋巴瘤亚型,每年影响约1500名患者。BL有三种形式(散发性、地方性、免疫缺陷相关型),地方性形式最初是在疟疾流行地区被发现由爱泼斯坦-巴尔病毒驱动。BL具有特征性的t8; 14细胞遗传学易位,导致MYC基因的组成性激活,从而驱动BL细胞分裂。BL的治疗已使许多患者治愈,但在BL治疗方法中,严重的毒性和与治疗相关的并发症仍然是个问题。复发和难治性疾病的治疗选择仍然有限,不过正在研究新的治疗方法来阻断MYC激活,这可能为复发和难治性疾病患者带来有前景的选择。