Ott M M, Ott G, Roblick U, Linke B, Kneba M, de Leon F, Müller-Hermelink H K
Institute of Pathology, University of Würzburg, Germany.
Leukemia. 1995 Apr;9(4):609-14.
Analyses for clonality in cases of Richter's syndrome have provided evidence for a clonal evolution of high-grade lymphoma in most patients, while in others an independent cellular clone seems to exist in the secondary neoplasm. Richter's syndrome with an isolated high-grade lymphoma of the stomach has been rarely reported in patients with pre-existing B cell chronic lymphocytic leukemia (CLL). We investigated four cases of CLL or lymphoplasmacytoid immunocytoma (LPIC) with development of a localized high-grade B cell lymphoma in the stomach. Southern blotting showed different rearrangements of the immunoglobulin light and heavy chain genes in the tumor cells of the low-grade lymphoma and the gastric tumor in two cases. Comparison of the DNA sequences of the CDR3 region of the immunoglobulin genes revealed different clones in another case. By means of chromosomal in situ hybridization, trisomy 3 was detected in two cases of high-grade lymphoma of the stomach, but not in the cells of the associated low-grade tumor. Our findings indicate that high-grade non-Hodgkin's lymphomas arising localized in the stomach of patients with CLL or immunocytoma are not clonally related to the pre-existing low-grade lymphoma and, therefore indeed, present true secondary neoplasms.
对里氏综合征病例的克隆性分析为大多数患者高级别淋巴瘤的克隆进化提供了证据，而在其他患者中，继发性肿瘤中似乎存在独立的细胞克隆。既往有B细胞慢性淋巴细胞白血病（CLL）的患者中，很少报告患有孤立性胃高级别淋巴瘤的里氏综合征。我们调查了4例CLL或淋巴浆细胞样免疫细胞瘤（LPIC）患者，其胃中出现了局限性高级别B细胞淋巴瘤。Southern印迹法显示，在2例患者的低级别淋巴瘤和胃肿瘤的肿瘤细胞中，免疫球蛋白轻链和重链基因存在不同的重排。对免疫球蛋白基因CDR3区域DNA序列的比较显示，另一例患者存在不同的克隆。通过染色体原位杂交，在2例胃高级别淋巴瘤病例中检测到三体3，但在相关低级别肿瘤的细胞中未检测到。我们的研究结果表明，CLL或免疫细胞瘤患者胃中出现的局限性高级别非霍奇金淋巴瘤与既往的低级别淋巴瘤不存在克隆相关性，因此确实是真正的继发性肿瘤。
