弥漫大B细胞淋巴瘤合并自身免疫性溶血性贫血的临床特点分析

Characteristics of diffuse large B-cell lymphoma combined with autoimmune hemolytic anemia

目的:探讨弥漫大B细胞淋巴瘤(diffuse large B-cell lymphoma,DLBCL)合并自身免疫性溶血性贫血(autoimmune hemolytic-anemia,AIHA)的临床特点,进一步提高对该病的认识。方法:回顾性分析2012年1月至2018年12月就诊于皖南医学院第一附属医院收治的6例DLBCL合并AIHA患者资料,分析其临床特征、实验室结果、治疗及转归。结果:6例患者中男性2例,女性4例;中位发病年龄60(49~73)岁。确诊时血红蛋白中位数69.5(55~85)g/L,网织红细胞比例中位数6.65%(1.2%~14.7%),Coombs试验全部为阳性。6例患者中Ann Arbor分期:Ⅲ期4例,Ⅳ期2例;IPI评分3分4例,4分1例,5分1例。AIHA发生于DLBCL之前2例,AIHA与DLBCL同时发生2例,AIHA发生于淋巴瘤确诊之后2例。4例患者联合化疗及激素治疗,2例患者单用化疗;其中3例患者存活,3例患者死亡,中位总生存期17个月。结论:DLBCL合并AIHA临床少见,治疗上应根据患者的发病特点、治疗疗效采取个性化的治疗方案,预后主要取决于DLBCL的缓解程度。

Objective:To investigate the clinical characteristics of diffuse large B-cell lymphoma(DLBCL)combined with autoimmune hemolytic anemia(AIHA).Methods:Data of six patients with DLBCL complicated by AIHA treated at the First Affiliated Hospital of Wannan Medical College between January 2012 and December 2018 were retrospectively reviewed.The clinical manifestations,laboratory results,treatment and follow-up results were analyzed.Results:Of these six patients with DLBCL complicated by AIHA,2 patients were males and 4 were females,aged between 49 and 73 years old,with a median age of 60 years old.Their median level of hemoglobin was 69.5(55-85)g/L,the medical ratio of reticulocytes was 6.65%(1.2%-14.7%),and the Coombs test positive rate was 100%.Of the 6 patients,4 presented with stageⅢand 2 stageⅣDLBCL.Four patients had an International Prognostic Index(IPI)of 3,1 was IPI 4,and 1 was IPI 5.The diagnosis of AIHA preceded that of DLBCL in 2 patients.Contrastingly,in 4 patients,AIHA occurred either when DLBCL was diagnosed or during the course of the disease.Four patients received prednisone between the chemotherapy intermittent period,and 2 patients received only chemotherapy.Of the 6 patients,3 passed away.The median overall survival(OS)time was 17 months.Conclusions:AIHA remains a rare occurrence in patients with DLBCL.The treatment schedule should be personalized based on the patient's onset status and therapeutic effect.Prognosis depends largely on the response status of the diffuse large B-cell lymphoma.