Parrens M, Sawan B, Dubus P, Lacombe F, Marit G, Vergier B, Reiffers J, de Mascarel A, Merlio J P
Department of Pathology, Hôpital du Haut-Lévêque, Pessac, France.
Mod Pathol. 2001 May;14(5):452-7. doi: 10.1038/modpathol.3880333.
The clinical and morphologic transformation of 3 to 5% of chronic lymphocytic leukemia (CLL) to diffuse large-cell lymphoma (DLCL) is commonly referred to as Richter's syndrome. Richter's syndrome occurs mostly in lymph nodes and may represent a second neoplasm or a transformation from the same clonal population. Clinical features in six patients with digestive Richter's syndrome were recorded. Paired samples of CLL and DLCL were investigated by immunohistological analysis (n = 6) and by polymerase chain reaction (PCR) for immunoglobulin heavy-chain gene rearrangement (n = 4). Histological examination revealed the involvement of the gastrointestinal tract by DLCL of B-cell phenotype (n = 6). The same monoclonal rearrangement between CLL and DLCL was demonstrated by PCR and sequencing analyses in two patients. The monoclonal rearrangement was different between CLL and DLCL in only one case. Median survival was 22 months for five patients receiving chemotherapy, suggesting that digestive Richter's syndrome has a better prognosis than nodal Richter's syndrome. Indeed, appropriate surgical resection combined with chemotherapy led to partial or complete remission in four patients.
3%至5%的慢性淋巴细胞白血病（CLL）向弥漫性大细胞淋巴瘤（DLCL）的临床和形态学转变通常被称为里氏综合征。里氏综合征大多发生于淋巴结，可能代表第二种肿瘤或源自同一克隆群体的转变。记录了6例消化型里氏综合征患者的临床特征。通过免疫组织学分析（n = 6）以及针对免疫球蛋白重链基因重排的聚合酶链反应（PCR）（n = 4）对CLL和DLCL的配对样本进行了研究。组织学检查显示胃肠道受到B细胞表型的DLCL侵犯（n = 6）。通过PCR和测序分析在2例患者中证实了CLL和DLCL之间存在相同的单克隆重排。仅在1例患者中CLL和DLCL之间的单克隆重排有所不同。5例接受化疗的患者的中位生存期为22个月，这表明消化型里氏综合征的预后比淋巴结型里氏综合征更好。确实，适当的手术切除联合化疗使4例患者实现了部分或完全缓解。
