套细胞淋巴瘤41例临床分析

Clinical Analysis of 41 Cases of Mantle Cell Lymphomas

目的了解套细胞淋巴瘤在中国人非霍奇金淋巴瘤中的构成比、临床特点及预后。方法收集在武汉协和医院就诊的312例非霍奇金淋巴瘤(NHL)患者淋巴组织标本,根据世界卫生组织(WHO)2001年淋巴瘤分类标准进行分型,运用多聚酶链反应检测NHL患者石蜡包埋的淋巴组织标本bcl-1/IgH基因重排;以免疫组织化学方法分析组织切片的细胞周期素D1表达。收集患者的临床资料,如年龄、临床分期、结外病变、血清乳酸脱氢酶水平、治疗效果等。结果在312例非霍奇金淋巴瘤患者中有41例(13%)病理诊断为套细胞淋巴瘤,套细胞淋巴瘤免疫分型均为B淋巴细胞型,并伴有bcl-1基因重排和(或)细胞周期D1蛋白高表达。这些套细胞淋巴瘤临床特点是患者多为老年男性、临床分期较晚、常合并结外病变,乳酸脱氢酶水平高,治疗效果不佳,预后差。结论套细胞淋巴瘤在中国人非霍奇金淋巴瘤中所占构成比(13%)较欧美白人(2%～8%)高。套细胞淋巴瘤治疗效果差,预后不佳,需要寻找新的治疗方法。

Objective To investigate the clinical characters and prognosis of mantle cell lymphomas （MCL） and its proportion in Chinese patients with non Hodgkin＇s lymphomas （NHL）. Methods Specimens of 312 cases diagnosed as NHL were colleted and typed according to 2001 WHO classification of lymphoid neoplasms, bcl-1 gene rearrangement was analyzed by polymerase chain reaction （PCR）, and the Cyclin D1 protein expression was detected by immunohistochemistry. The clinical data of 312 patients were collected, including age, clinical stage, extra nodal lesions, serum level of lactate dehydrogenase and curative effectiveness. Results Among 312 cases of NHL, 41 （13%） were diagnosed as MCL （immune phenotype： B-cell type） with bcl-1 gene rearrangement and（or） Cyclin D1 protein overexpression. The MCL was clinically characterized by higher international prognostic index （IPI） and lower complete remission （CR） than other NHL patients. Conclusion The proportion （13%） of MCL in Chinese patients with NHL is higher than that （2%-8%） in Euramerican. The curative efficacy of MCL and prognosis are very poor.