Gaidano G, Pastore C, Lanza C, Mazza U, Saglio G
Department of Biomedical Sciences and Human Oncology, University of Turin, Ospedale San Luigi Gonzaga, Italy.
Ann Hematol. 1994 Dec;69(6):281-90. doi: 10.1007/BF01696556.
A high frequency of lymphoma in human immunodeficiency virus-infected individuals has been reported since the outbreak of the acquired immunodeficiency syndrome (AIDS) epidemic in 1982. AIDS-associated non-Hodgkin's lymphoma (AIDS-NHL) is almost invariably derived from B cells and is classified as high- or intermediate-grade NHL, according to the working formulation. Two main histologic types are recognized, including small noncleaved cell lymphoma (SNCCL) and diffuse large cell lymphoma (DLCL). Pre-existing host factors putatively involved in lymphoma development include disrupted immunosurveillance, deregulated cytokine production, chronic antigen stimulation, and infection by Epstein-Barr virus (EBV). These alterations are associated with the development of multiple oligoclonal expansions which correspond to the clinical phase known as persistent generalized lymphadenopathy (PGL). The appearance of a true AIDS-NHL is characterized by the presence of a monoclonal B-cell population displaying several genetic lesions, including monoclonal EBV infection, c-MYC and BCL-6 rearrangements, RAS mutations, p53 inactivation, and 6q deletions. These genetic lesions cluster into two distinct molecular pathways, which specifically associate with the different histologic subtypes of AIDS-NHL, i.e., AIDS-SNCCL and AIDS-DLCL. The presence of distinct genetic pathways for AIDS-SNCCL and AIDS-DLCL correlate with a number of clinical features which distinguish these two groups of tumors, including differences in the age of onset, CD4 counts at the time of presentation, time elapsed since HIV infection, and clinical outcome.
自1982年获得性免疫缺陷综合征（AIDS）流行爆发以来，已有报道称人类免疫缺陷病毒感染个体中淋巴瘤的发病率较高。艾滋病相关非霍奇金淋巴瘤（AIDS-NHL）几乎均源自B细胞，根据工作分类法，被归类为高级别或中级别NHL。主要识别出两种组织学类型，包括小无裂细胞淋巴瘤（SNCCL）和弥漫大细胞淋巴瘤（DLCL）。推测参与淋巴瘤发生的宿主因素包括免疫监视破坏、细胞因子产生失调、慢性抗原刺激以及爱泼斯坦-巴尔病毒（EBV）感染。这些改变与多个寡克隆扩增的发展相关，这些扩增对应于称为持续性全身性淋巴结病（PGL）的临床阶段。真正的AIDS-NHL的出现特征是存在显示多种基因损伤的单克隆B细胞群体，包括单克隆EBV感染、c-MYC和BCL-6重排、RAS突变、p53失活和6q缺失。这些基因损伤聚集成两个不同的分子途径，它们分别与AIDS-NHL的不同组织学亚型，即AIDS-SNCCL和AIDS-DLCL相关。AIDS-SNCCL和AIDS-DLCL不同基因途径的存在与区分这两组肿瘤的许多临床特征相关，包括发病年龄、就诊时的CD4计数、自HIV感染以来经过的时间以及临床结局的差异。
