Rechavi G, Ben-Bassat I, Berkowicz M, Martinowitz U, Brok-Simoni F, Neumann Y, Vansover A, Gotlieb-Stematsky T, Ramot B
Institute of Hematology, Chaim Sheba Medical Center, Tel-Hashomer, Israel.
Blood. 1987 Dec;70(6):1713-7.
In two hemophilic brothers infected by the human immunodeficiency virus (HIV), Burkitt's leukemia developed within 1 year. Both patients were treated by aggressive chemotherapy, and both are still in complete remission for 23 and 14 months, respectively. Sera from both brothers contained anti-HIV antibodies. However, DNA extracted from the tumor cells, when analyzed by Southern blot using a cloned HIV probe, did not reveal HIV-related sequences. Hybridization experiments with an Epstein-Barr virus (EBV) probe revealed the presence of EBV-specific sequences in the tumors' DNA. In both patients' tumors rearranged c-myc genes were found. The rearrangements occurred in both genes 3' to the third exon of c-myc, thereby suggesting that a variant chromosomal translocation took place in both cases. Indeed, karyotype analysis of the malignant cells of one of the patients revealed the variant t(2:8) translocation. In contrast to the majority of Burkitt's tumors carrying this translocation, which are kappa light-chain producers, cells of our patient expressed lambda chains. Furthermore, in both cases the lymphoblasts carried IgG on the surface, again an unusual finding in Burkitt's tumors. Finally, because both patients had an identical HLA phenotype, the role of genetic factors in the development of such tumors should be considered.
在两名感染人类免疫缺陷病毒（HIV）的血友病兄弟中，1年内发生了伯基特淋巴瘤。两名患者均接受了积极的化疗，目前分别已完全缓解23个月和14个月。两名兄弟的血清中均含有抗HIV抗体。然而，用克隆的HIV探针通过Southern印迹法分析从肿瘤细胞中提取的DNA时，未发现与HIV相关的序列。用爱泼斯坦-巴尔病毒（EBV）探针进行的杂交实验表明，肿瘤DNA中存在EBV特异性序列。在两名患者的肿瘤中均发现了重排的c-myc基因。重排发生在c-myc第三个外显子下游的两个基因中，从而表明在这两种情况下都发生了变异的染色体易位。实际上，对其中一名患者的恶性细胞进行核型分析发现了变异的t(2;8)易位。与大多数携带这种易位的伯基特肿瘤产生κ轻链不同，我们患者的细胞表达λ链。此外，在这两种情况下，淋巴母细胞表面都携带IgG，这在伯基特肿瘤中也是一个不寻常的发现。最后，由于两名患者具有相同的HLA表型，因此应考虑遗传因素在这类肿瘤发生中的作用。
