Ambinder R F, Griffin C A
Johns Hopkins Oncology Center, Baltimore, Maryland.
Curr Opin Oncol. 1991 Oct;3(5):806-12. doi: 10.1097/00001622-199110000-00002.
Correlations between cytogenetics, histology, and clinical course continue to emerge in studies of non-Hodgkin's lymphomas. The previously recognized association between the t(14;18) chromosomal translocation and follicular lymphoma has been confirmed; abnormalities of chromosome 3 have correlated specifically with diffuse large cell lymphoma and abnormalities of chromosome 1 have been frequently present in T-cell lymphomas. Rearrangements involving 11q13 (bcl-1) occur most commonly in diffuse lymphocytic lymphoma of intermediate differentiation. Several new recurrent chromosomal abnormalities have also been described. The molecular fine structure of the t(8-14) chromosomal translocation in Burkitt's lymphoma appears to differ between endemic (Epstein-Barr virus-associated) and sporadic cases. In endemic Burkitt's lymphomas, the chromosomal breakpoint is usually far upstream of c-myc oncogene, leaving the regulatory region of the gene intact. In sporadic tumors, a large part of the regulatory region is separated from the gene and transcription is initiated at sites within the first intron. These data raise the possibility that Epstein-Barr virus may contribute to the deregulation of the c-myc gene and that this interaction may be required for tumorigenesis in the presence of some, but not all, types of c-myc damage arising from chromosomal translocations. Partner proteins that oligomerize with c-Myc have been identified in humans and mice (Max and Myn). The partners share with c-Myc the DNA-binding and coiled-coil motifs that are recognized in many other proteins and that function as transcriptional regulators. The Bcl-2 protein has been shown to be a mitochondrial inner membrane protein that blocks programmed cell death (apoptosis). Viral expression has been demonstrated in Epstein-Barr virus-associated Hodgkin's disease, and the spectrum of Epstein-Barr virus-associated lymphoproliferative disease has been expanded to include some T-cell malignancies. A new human herpesvirus has been associated with some cases of Hodgkin's disease.
在非霍奇金淋巴瘤的研究中,细胞遗传学、组织学与临床病程之间的相关性不断显现。先前已确认的t(14;18)染色体易位与滤泡性淋巴瘤之间的关联得到了证实;3号染色体异常与弥漫性大细胞淋巴瘤具有特异性相关性,1号染色体异常则常见于T细胞淋巴瘤。涉及11q13(bcl-1)的重排最常出现在中度分化的弥漫性淋巴细胞淋巴瘤中。还描述了几种新的复发性染色体异常。伯基特淋巴瘤中t(8;14)染色体易位的分子精细结构在地方性(与爱泼斯坦-巴尔病毒相关)和散发性病例中似乎有所不同。在地方性伯基特淋巴瘤中,染色体断点通常位于c-myc癌基因的上游很远位置,使该基因的调控区域保持完整。在散发性肿瘤中,大部分调控区域与该基因分离,转录在第一个内含子内的位点起始。这些数据提示,爱泼斯坦-巴尔病毒可能导致c-myc基因失调,并且在存在某些(但并非全部)由染色体易位引起的c-myc损伤类型时,这种相互作用可能是肿瘤发生所必需的。在人类和小鼠中已鉴定出与c-Myc形成寡聚体的伙伴蛋白(Max和Myn)。这些伙伴蛋白与c-Myc共享在许多其他蛋白质中也存在的DNA结合和卷曲螺旋基序,这些基序作为转录调节因子发挥作用。Bcl-2蛋白已被证明是一种线粒体内膜蛋白,可阻止程序性细胞死亡(凋亡)。在与爱泼斯坦-巴尔病毒相关的霍奇金病中已证实有病毒表达,并且与爱泼斯坦-巴尔病毒相关的淋巴增殖性疾病谱已扩大到包括一些T细胞恶性肿瘤。一种新的人类疱疹病毒已与某些霍奇金病病例相关联。