Bornkamm G W, Polack A, Eick D, Berger R, Lenoir G M
Institut für Medizinische Mikrobiologie und Hygiene, Universität Freiburg.
Onkologie. 1987 Aug;10(4):196-204. doi: 10.1159/000216407.
Burkitt's lymphoma is characterized by particular epidemiological features. It is a frequent childhood tumor in children in tropical Africa and occurs at a much lesser frequency all over the world. Chromosomal translocation affecting the long arm of chromosome 8 (band 8q24) and one of the chromosomes carrying the immunoglobulin loci (chromosomes 2, 14 or 22) are regularly observed in Burkitt's lymphoma, regardless of whether the tumor occurred in high or low incidence areas. The prevalence of Burkitt's lymphoma in Africa appears to be related to two factors: holo- or hyperendemic malaria and presence of Epstein-Barr virus genomes in the tumor cells. We present a model of pathogenesis, in which stimulation of B cells by malaria is the primary event in the development of the disease. The risk of the chromosomal translocation should be increased by increasing the number of new B cells generated per time. According to our model, the translocation leads to constitutive c-myc activation and makes the cells responsive to growth factors without inducing proliferation on its own. Infection of a translocation-carrying cell with EBV may provide an additional growth advantage and drive the cell further towards a fully malignant state.
伯基特淋巴瘤具有特定的流行病学特征。它是热带非洲儿童中常见的儿童肿瘤，在世界各地的发病率要低得多。在伯基特淋巴瘤中经常观察到影响8号染色体长臂（8q24带）和携带免疫球蛋白基因座的染色体之一（2号、14号或22号染色体）的染色体易位，无论肿瘤发生在高发病率地区还是低发病率地区。非洲伯基特淋巴瘤的流行似乎与两个因素有关：全地方性或高度地方性疟疾以及肿瘤细胞中爱泼斯坦-巴尔病毒基因组的存在。我们提出了一种发病机制模型，其中疟疾对B细胞的刺激是该疾病发展的主要事件。每次产生的新B细胞数量增加应会增加染色体易位的风险。根据我们的模型，易位导致c-myc组成性激活，并使细胞对生长因子有反应，但自身不诱导增殖。携带易位的细胞感染EBV可能会提供额外的生长优势，并使细胞进一步发展为完全恶性状态。
