Ansell S M, Li C Y, Lloyd R V, Phyliky R L
Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
Am J Hematol. 1999 Feb;60(2):99-104. doi: 10.1002/(sici)1096-8652(199902)60:2<99::aid-ajh3>3.0.co;2-t.
Chronic lymphocytic leukemia (CLL) may convert to a diffuse large cell lymphoma (Richter's syndrome) over time. In occasional cases of Richter's transformation, Epstein-Barr virus (EBV) has been identified in the lymphoma cells. To evaluate the association of EBV infection with Richter's syndrome, the biopsy specimens and clinical records of 25 patients who were seen at the Mayo Clinic between 1984-1996 were retrospectively evaluated for the presence of EBV by immunoperoxidase staining for expression of EBV latent membrane protein (LMP), as well as the expression of EBV RNA and DNA in the cells by in situ hybridization. Four of the 25 patients showed evidence of EBV in the diffuse large cell lymphoma cells-three patients with a B-cell phenotype were positive for LMP, EBV DNA, and RNA; and one patient with a T-cell phenotype had positive EBV RNA in the large cell lymphoma cells. The Richter's syndrome was treated with combination chemotherapy in 15 patients, three received radiotherapy, three were followed without further therapy after a splenectomy, two died before treatment could be started, and one patient had insufficient follow-up. One patient with evidence of EBV in large cell lymphoma cells was treated with acyclovir as initial therapy. The median survival of EBV-positive patients was three months compared with nine months for EBV-negative patients, but this difference was not statistically significant (P = 0.385). Evidence for EBV infection related to Richter's transformation was present in 16% of the patients in this study and may be associated with a poorer outcome. Primary therapy with acyclovir in one patient did not seem to be beneficial and other therapeutic modalities in patients with EBV-positive Richter's transformation need to be explored.
慢性淋巴细胞白血病（CLL）随着时间推移可能会转化为弥漫性大细胞淋巴瘤（里氏综合征）。在偶发的里氏转化病例中，已在淋巴瘤细胞中鉴定出爱泼斯坦-巴尔病毒（EBV）。为评估EBV感染与里氏综合征的关联，对1984年至1996年间在梅奥诊所就诊的25例患者的活检标本和临床记录进行了回顾性评估，通过免疫过氧化物酶染色检测EBV潜伏膜蛋白（LMP）的表达来确定EBV的存在，同时通过原位杂交检测细胞中EBV RNA和DNA的表达。25例患者中有4例在弥漫性大细胞淋巴瘤细胞中显示出EBV感染的证据——3例B细胞表型患者的LMP、EBV DNA和RNA呈阳性；1例T细胞表型患者的大细胞淋巴瘤细胞中EBV RNA呈阳性。15例里氏综合征患者接受了联合化疗，3例接受了放疗，3例在脾切除术后未进一步治疗进行随访，2例在开始治疗前死亡，1例患者随访不足。1例大细胞淋巴瘤细胞中有EBV感染证据的患者接受了阿昔洛韦作为初始治疗。EBV阳性患者的中位生存期为3个月，而EBV阴性患者为9个月，但这种差异无统计学意义（P = 0.385）。本研究中16%的患者存在与里氏转化相关的EBV感染证据，且可能与较差的预后相关。1例患者接受阿昔洛韦作为初始治疗似乎并无益处，需要探索针对EBV阳性里氏转化患者的其他治疗方式。
