Tamaru J, Hummel M, Marafioti T, Kalvelage B, Leoncini L, Minacci C, Tosi P, Wright D, Stein H
Institute of Pathology, Klinikum Benjamin Franklin, Free University Berlin.
Am J Pathol. 1995 Nov;147(5):1398-407.
The normal counterpart of the neoplastic B cells occurring in Burkitt's lymphomas (BL) is an issue of controversial debate. To clarify this matter, a semi-nested primer polymerase chain reaction was performed to amplify the VDJ rearrangements of the immunoglobulin heavy chain (VH) gene of DNA extracts from 10 (8 sporadic and 2 endemic) BL cases. The resulting amplificates were sequenced for comparison with known germ line VH segments. The control cases comprised six cases of B cell chronic lymphocytic leukemia and six cases of mantle cell lymphoma known to display naive nonmutated, ie, pre-germinal center VH configurations; and eight cases of follicular center lymphoma known to display mutated VH genes with signs of a still-ongoing mutation reaction, characteristic for germinal center cells and lymphomas that derive therefrom. The results of this approach revealed that both sporadic and endemic BL express mutated VH genes with a mutation frequency considerably lower (4.9% and 5.4%, respectively) than that observed in follicular center lymphoma (11.8%). In addition, after subcloning the amplificates, sequence analysis revealed no signs of ongoing mutations. These results led us to conclude that the derivation of neoplastic B cells in BL is definitely not from naive, nonmutated pre-germinal center B cells. Instead, our findings support the view that BL cells stem either from early centroblasts that are arrested after an initial hypermutation reaction, or from germinal center B cells that have differentiated in terms of surface immunoglobulin profile and mutation pattern but not in terms of morphology and proliferation toward SIgM+ IgD- memory B cells because of the deregulated c-myc gene expression.
伯基特淋巴瘤(BL)中出现的肿瘤性B细胞的正常对应物是一个有争议的问题。为了阐明这一问题,我们进行了半巢式引物聚合酶链反应,以扩增10例(8例散发性和2例地方性)BL病例DNA提取物中免疫球蛋白重链(VH)基因的VDJ重排。对所得扩增产物进行测序,以便与已知的胚系VH区段进行比较。对照病例包括6例B细胞慢性淋巴细胞白血病和6例套细胞淋巴瘤,已知它们显示出未发生突变的幼稚型,即生发中心前VH构型;以及8例滤泡中心淋巴瘤,已知它们显示出具有仍在进行的突变反应迹象的突变VH基因,这是生发中心细胞及由此衍生的淋巴瘤的特征。该方法的结果显示,散发性和地方性BL均表达突变的VH基因,其突变频率(分别为4.9%和5.4%)明显低于滤泡中心淋巴瘤(11.8%)。此外,在对扩增产物进行亚克隆后,序列分析未发现持续突变的迹象。这些结果使我们得出结论,BL中肿瘤性B细胞肯定不是来自未发生突变的幼稚生发中心前B细胞。相反,我们的研究结果支持这样一种观点,即BL细胞要么源自初始超突变反应后停滞的早期中心母细胞,要么源自由于c-myc基因表达失调而在表面免疫球蛋白谱和突变模式方面发生分化,但在形态和增殖方面未分化为SIgM+IgD-记忆B细胞的生发中心B细胞。