首发症状Coombs试验阴性自身免疫性溶血性贫血的非何杰金淋巴瘤

摘要

The cases of lymphoma accompanied or preceded by Coombs' test positive autoimmune hemolytic anemia (AIHA) have been reported.However,Coombs' test negative AIHA prior to the diagnosis of lymphoma was rarely described.Herein,this article reports a case of non-Hodgkin's lymphoma (NHL) preceded about 1.5 years by Coombs test negative AIHA.A woman aged 69 was diagnosed with HA based on the history and laboratory tests.Further studies revealed that this patient was negative with Coombs' test for IgG,IgM,IgA and C3.After all possible causes of HA,especially malignancies were ruled out,the patient was diagnosed with Coombs' test negative AIHA and treated with prednisolone.The patient responded well initially to steroid treatment.Two recurrences of acute HA were presented at time of 10 months post steroid cessation,and immediately after an attempt to withdraw steroid,respectively,but the hemolysis was effectively controlled by reinstitution of prednisolone.At third recurrence,however,the patient was no longer responding to steroid,and was found with cervical lymphadenopathy.Coombs' test for IgG,IgM,IgA and C3 remained negative.B cell NHL was diagnosed by pathology.After receiving 6 cycles of CHOP chemotherapy,the patient was lymphoma free,but the hemolysis was not improved,however,which was effectively controlled by the following low dose-rituximab(RTX) therapy.The patient was still kept in a remission of lymphoma free of anemia.In conclusion,this report presented a very rare case of NHL with Coombs' test negative AIHA as initial major clinical manifestation.%非何杰金淋巴瘤(NHL)伴发自身免疫性溶血性贫血(AIHA)或AIHA发生在NHL诊断之前或治疗过程中已有不少报道,但以Coombs试验阴性AIHA为首发症状的NHL鲜有报道.在此,本文报道1例1.5年反复溶血发作的Coombs试验阴性AIHA后合并NHL的患者.患者女性,69岁,根据病史和实验室检查诊断为Coombs试验阴性AIHA,给予强的松治疗后血红蛋白恢复正常,停药后溶血反复复发2次,强的松治疗仍有效.第3次复发时强的松治疗无效,并出现颈部淋巴结肿大,病理检查确诊为NHL.给予6个疗程的CHOP方案化疗,NHL治愈,但溶血仍不能控制,给予小剂量利妥昔单克隆抗体( rituximab,RTX)治疗后,溶血很快停止,此后给予3次小剂量RTX进行维持治疗,NHL和AIHA呈持续缓解状态.结论:本文报告了一例十分罕见的非何杰金淋巴瘤,其发病时的主要临床症状为Coombs阴性自身免疫性溶血性贫血.