45例间变性大细胞淋巴瘤临床病理特点及疗效分析

Analysis of Clinicopathological Characters and Outcomes of 45 Patients with Anaplastic Large Cell Lymphoma

目的分析间变性大细胞淋巴瘤(ALCL)的临床病理特点、远期效果和影响预后的因素。方法回顾性分析2000年1月—2008年12月我院收治的45例ALCL患者的临床资料,并分析远期疗效,用Cox模型分析预后因素。结果原发皮肤和系统性ALCL分别有7例(84.4%)和38例(15.6%)。20例(44.4%)伴有B症状者,乳酸脱氢酶升高者8例(21.6%),低危组、中低危组共31例(68.9%),28例(62.2%)ALK阳性。26例(57.8%)为T细胞来源,6例(13.3%)为B细胞来源,11例(22.2%)T细胞和B细胞标记均为阴性,为null型,2例T细胞和B细胞标记均为阳性。原发皮肤和系统性ALCL治疗后的有效率分别为100%和92.1%,5年OS率分别为83.3%和63.9%(χ2=0.707,P=0.401)。ALK阳性和阴性患者的5年OS率分别87.5%和46.9%(χ2=10.992,P=0.01)。单因素分析ALK表达状况(P=0.01)、国际预后指数评分(P=0.000)、临床分期(P=0.005)对生存期的影响有统计学意义,ALK的表达状况(P=0.012)和国际预后指数评分(P=0.000)是影响总生存期的独立因素。结论 ALCL中约80%为原发系统性,近期和远期疗效较好,约60%的患者ALK阳性,ALK和国际预后指数为独立的预后因素。

Objective To investigate the factors affect on the clinicopathological characters,the outcome treatment and the prognostic of anaplastic large cell lymphoma（ALCL）.Methods The clinical data of 45 patients with ALCL who received treatment in our hospital from Jan 2000 to Dec 2008 were analyzed retrospectively.The survival was estimated by Kaplan-Meier and the prognostic factors were analyzed with Cox model.Results 84.4%（38 patients） and 15.6%（7 patients） were primary cutaneous and systemic ALCL respectively.Twenty patients（44.4%） manifested with systemic symptoms and 8 patients（21.6%）with elevated level of lactate dehydrogenase.According to the International Prognostic Index（IPI） of Non-Hodgkin lymphoma,31 patients（68.9%）were the low and low-intermediate risk.And anaplstic lymphoma kinase（ALK） was positive in 28 patients（62.2%）.26 patients（57.8%） were T-cell origin,6 ones（13.3%） were B-cell origin markers 11 ones（22.2%）were null type and both the T-cell and B-cell markers were positive in the other two patients（4.4%）.The response rates,5-year overall survival rates of primary cutaneous and systemic ALCL were 100% and 92.1%,83.3% and 63.9%（χ2=0.707,P=0.401） respectively.The 5-year overall survival rates of patients with positive and negative ALK were 87.5% and 46.9%（χ2=10.992,P=0.01）.With the univariate analysis,the expression of ALK（P=0.01）,the score of IPI（P=0.000） and the clinical staging（P=0.005） were the prognostic factors affect the overall survival.With multivariate analysis,both ALK（0.012） and IPI（P=0.000） were the independent prognostic factors.Conclusion Approximately,80% of ALCL are primary systemic and 60% of patients express the ALK.Both the response and the survival are satisfied.The ALK and IPI score are the independent prognostic factors affect the overall survival.