Casulo Carla, Friedberg Jonathan
Wilmot Cancer Institute, University of Rochester, Rochester, NY, USA,
Curr Hematol Malig Rep. 2015 Sep;10(3):266-71. doi: 10.1007/s11899-015-0263-4.
Burkitt lymphoma is an uncommon form of aggressive lymphoma affecting approximately 1200 patients per year in the USA. It is characterized by a translocation involving the MYC oncogene. Three subtypes of Burkitt lymphoma are recognized: the endemic form, occurring primarily in Africa and associated with the Epstein-Barr virus (EBV); the sporadic form, representing less than 3 % of all non-Hodgkin lymphomas (NHL); and the immunodeficiency-associated form, occurring primarily in HIV-infected patients. Burkitt lymphoma appears histologically with a diffuse pattern of intermediate-sized monomorphic B cells, multiple nucleoli, a very high proliferative rate, and frequent mitotic figures. Recent advances in transcriptional profiling have improved the current molecular understanding of Burkitt lymphoma and have better characterized its mutational landscape. Most Burkitt lymphoma patients are cured with intensive treatment; however, prognosis is poor in elderly patients and those with relapsed disease.
伯基特淋巴瘤是侵袭性淋巴瘤的一种罕见形式,在美国每年约影响1200名患者。其特征是涉及MYC癌基因的易位。伯基特淋巴瘤有三种亚型:地方性亚型,主要发生在非洲,与爱泼斯坦-巴尔病毒(EBV)相关;散发型,占所有非霍奇金淋巴瘤(NHL)的比例不到3%;免疫缺陷相关型,主要发生在感染HIV的患者中。伯基特淋巴瘤在组织学上表现为中等大小单形性B细胞的弥漫性模式、多个核仁、非常高的增殖率和频繁的有丝分裂象。转录谱分析的最新进展改善了目前对伯基特淋巴瘤的分子理解,并更好地描绘了其突变格局。大多数伯基特淋巴瘤患者通过强化治疗得以治愈;然而,老年患者和复发患者的预后较差。