McDonnell J M, Beschorner W E, Staal S P, Spivak J L, Mann R B
Cancer. 1986 Nov 1;58(9):2031-7. doi: 10.1002/1097-0142(19861101)58:9<2031::aid-cncr2820580913>3.0.co;2-j.
The diagnoses of chronic lymphocytic leukemia (CLL) in prolymphocytic transformation, and diffuse large cell lymphoma (DLC), were made simultaneously in a 71-year-old man. The DLC showed mu lambda surface immunoglobulin. The CLL in a lymph node and in the peripheral blood showed mu kappa. Immunoglobulin gene DNA analysis confirmed the presence of different rearrangements in the heavy and light chain genes of the CLL and DLC. Other cases reported of Richter's syndrome are discussed, and it is concluded that there may be two types of Richter's syndrome, those arising from transformation of a single clone, and those occurring from expansion of two morphologically and immunologically distinct clones, as, it is believed, is the case in this patient.
一名71岁男性同时被诊断为处于幼淋巴细胞转化期的慢性淋巴细胞白血病(CLL)和弥漫性大细胞淋巴瘤(DLC)。DLC显示μλ表面免疫球蛋白。淋巴结和外周血中的CLL显示μκ。免疫球蛋白基因DNA分析证实CLL和DLC的重链和轻链基因存在不同的重排。文中讨论了其他报道的里氏综合征病例,并得出结论,可能存在两种类型的里氏综合征,一种由单个克隆转化引起,另一种由两个形态学和免疫学上不同的克隆扩增引起,本文患者被认为属于后一种情况。