TAFRO综合征6例临床分析  

Clinical analysis of six cases of TAFRO syndrome

作　　者：刘素苗 郭乾育 乔鹏燕 崔银风 许珂 张莉芸 Liu Sumiao;Guo Qianyu;Qiao Pengyan;Cui Yinfeng;Xu Ke;Zhang Liyun(Department of Rheumatology and Immunology,Shanxi Bethune Hospital(Shanxi Academy of Medical Sciences,Tongji Shanxi Hospital),the Third Hospital of Shanxi Medical University,Taiyuan 030032,China;Shanxi Province Clinical Research Center for Dermatologic and Immunologic Diseases(Rheumatic Diseases),Taiyuan 030032,China)

机构地区：[1]山西白求恩医院(山西医学科学院,同济山西医院),山西医科大学第三医院风湿免疫科,太原030032 [2]山西省皮肤与免疫疾病(风湿病)临床医学研究中心,太原030032

出　　处：《中华风湿病学杂志》2025年第2期118-122,共5页Chinese Journal of Rheumatology

基　　金：山西白求恩医院科研攻关创新团队计划项目(2024AOXIANG02)。

摘　　要：分析TAFRO综合征的临床病理学特征,了解其与自身免疫病的异同点,进行正确的鉴别诊断。方法收集2014年1月至2022年3月在山西白求恩医院确诊为TAFRO综合征的患者6例,分析其临床、检验、病理、治疗等。结果6例患者中男性4例,女性2例,平均年龄(57.5±9.8)岁。6例患者均出现发热、水肿(包括胸、腹腔积液和全身性水肿)、血小板减少3项主要标准以及2项以上的次要标准。6例患者ESR、CRP均明显升高。1例IgA、IgG升高(IgA 4.10 g/L,IgG 19.05 g/L),1例IgG升高(IgG 19.33 g/L),3例正常,1例未测;血清IgG4:4例均阴性,2例未测;自身抗体:4例ANA阳性,其中1例合并抗SSA/Ro52抗体(+),抗SSA/Ro60抗体(+),抗SSB抗体(+),1例合并抗SSA/Ro60抗体(+),2例未测。6例均行骨髓细胞学检查,均为增生活跃,2例提示巨核细胞升高,其中1例伴间质纤维化;淋巴结病理学检查:5例符合Castleman病样的淋巴结病理表现,1例提示淋巴结反应性增生。结论尽管TAFRO综合征的诊断标准需要排除自身免疫病,但TAFRO综合征与自身免疫性疾病可以并存,且合并有TAFRO综合征样的结缔组织病有其特异的临床特点及治疗方案,临床上需要鉴别。ObjectiveThe clinicalpathological features of TAFRO syndrome were analyzed to clarify the similarities and differences between TAFRO syndrome and autoimmune diseases and to establish differential diagnosis.MethodsSix patients diagnosed with TAFRO syndrome in Shanxi Bethune Hospital from January 2014 to March 2022 were collected.The clinical,examination,pathology and treatment of TAFRO syndrome were analyzed and compared with autoimmune diseases,especially systemic lupus erythematosus and Sjögren′s syndrome.ResultsAmong the 6 patients,4 were males and 2 were females,with an average age of(57.5±9.8)years.All the 6 patients had fever,edema(including chest and abdominal effusion and systemic edema),thrombocytopenia(3 main criteria)and more than 2 secondary criteria.ESR and CRP were significantly elevated in 6 patients.There were 1 case of elevated IgA and IgG(IgA 4.10 g/L,IgG19.05 g/L),1 case of elevated igg(IgG 19.33 g/L),3 cases of normal and 1 case of undetected.Serum IgG4 was negative in 4 cases and undetected in 2 cases.Autoantibodies:4 cases were ANA positive,including 1 case with anti-SSA/Ro52(+),anti-SSA/Ro60(+),anti-SSB(+),1 case with anti-SSA/Ro60(+),and 2 untested.Bone marrow cytological examination was performed in 6 cases,all of which showed active hyperplasia,2 cases showed elevated megakaryocytes,and 1 case was accompanied by interstitial fibrosis.Pathological examination of lymph nodes:5 cases were consistent with Castleman′s disease,and 1 case was suggestive of reactive hyperplasia of lymph nodes.ConclusionAlthough the diagnostic criteria of TAFRO syndrome should exclude autoimmune diseases,TAFRO syndrome and autoimmune diseases can coexist,and the connective tissue disease complicated with TAFRO syndrome has its specific clinical characteristics and treatment plan,which needs to be identified clinically.

关 键 词：TAFRO综合征 自身免疫疾病 结缔组织病 

分 类 号：R593.2[医药卫生—内科学]