摘要目的 分析原发系统型间变性大细胞淋巴瘤(S-ALCL)的临床特点和预后相关因素.方法 回顾性分析北京大学医学部病理学系淋巴瘤研究室确诊的56例S-ALCL的临床资料,采用免疫组织化学SP法检测间变淋巴瘤激酶(ALK)和bel-2蛋白的表达情况.结果 56例S-ALCL患者中,中位年龄17岁,男女比例为1.67:1.预后分析可追访病例49例,死亡16例(32.65%),均在2年内死亡,3年和5年生存率均为64.28%.56例患者均进行了ALK和bcl-2的检测,其阳性率分别为73.21%和17.86%.单因素预后分析显示不同临床分期、是否伴有或结外发病和ALK是否阳性对患者总体生存率的影响差异有统计学意义.临床分期是影响患者长期生存的独立预后因素.结论 S-ALCL以40岁以下中青年男性发病为主,发病后第1年为死亡高发时段,对化疗敏感患者多数能达到完全缓解并获得长期生存.临床分期、伴有或结外发病、ALK对预测患者长期生存和指导治疗有重要意义.

abstractsObjective To study clinical characteristics and prognostic factors in primary systemic anaplastic large cell lymphoma (S-ALCL). Methods Clinical data of 56 S-ALCL were retrospectively analysed, who were diagnosed in Lymphoma Lab of Peking University Health Science Centre. Immunohistochemical staining for ALK-1 and bcl-2 were performed by standard SP method. Results The median age of patients is 17 years, and the ratio of sex was1.67:1 (male : female) in 56 cases of S-ALCL. Among of the 49 cases who were followed up, 32.65 % (16/49) of patients died, and all of them died within two years after diagnosis. The 3-year and 5-year overall survival were 64.28 %. 41 out of 56 cases (73.21 %) was positive for ALK-1 protein, while 10 cases out of 56 S-ALCL cases (17.86 %) positive for bcl-2. Clinical staging, extranodal sites of involvement or with extranodal sites of involvement and ALK were important prognostic factors with statistic significance by Long-rank test. Among of them, Clinical staging was the most independent prognostic factor by COX multivariate analysis. Conclusion S-ALCL was mostly seen in the young and middle-aged male patients. The death were most frequently occurred within two years after diagnosis. Most of the patients who have good responses to chemotherapy can get the complete remission and long-term survival. Clinical staging, extranodal sites of involvement or with extranodal sites of involvement and ALK were very important prognostic factors which can be used to predict the patients long term survival, and guide the treatment.