TAFRO综合征六例临床特征及诊疗分析  

A report of six TAFRO syndrome:clinical characteristics,diagnosis and treatment analysis

作　　者：吴春波[1] 张海燕[1] 邵诗幻 窦丽稳[1] 周倩云[1] 刘扬[2] 高伟波[1] 朱继红[1] Wu Chunbo;Zhang Haiyan;Shao Shihuan;Dou Liwen;Zhou Qianyun;Liu Yang;Gao Weibo;Zhu Jihong(Department of Emergency Peking University People′s Hospital,Beijing 100044,China;Department of Hematology,Peking University People′s Hospital,Beijing 100044,China)

机构地区：[1]北京大学人民医院急诊科,北京100044 [2]北京大学人民医院血液科,北京100044

出　　处：《中华医学杂志》2020年第8期624-628,共5页National Medical Journal of China

摘　　要：目的:探讨TAFRO综合征的临床特征、治疗及预后。方法:收集2011年12月至2019年4月间北京大学人民医院病理明确诊断Castleman病患者31例,分析其中符合TAFRO综合征的6例病例的临床资料,结合国内外文献,总结该病的临床表现、实验室检查、病理学特点、治疗方案及预后。结果:纳入TAFRO综合征诊断的患者6例(男5例),平均年龄41.5岁(27~59岁)。患者表现为急性或亚急性起病,发热、血小板减少、多浆膜腔积液、器官肿大和(或)全身水肿、肾功能损害。1例合并噬血细胞综合征,1例合并甲状腺功能低下,6例(6/6)白细胞介素6均升高,4例行血管内皮生长因子检查均升高,6例HIV均阴性,4例行人类疱疹病毒8检查均阴性,6例病理诊断中浆细胞型3例,混合型2例,透明血管型1例。2例患者行肾脏活检,1例符合血栓性微血管病肾损害伴亚急性肾小管间质肾炎,另1例为继发性毛细血管内增生性肾小球肾炎。2例行CHOP方案化疗,1例糖皮质激素治疗,3例糖皮质激素联合单抗类药物治疗,其中1例5年后死于病情进展,另5例患者病情稳定。结论:TAFRO综合征发病罕见,临床表现为多系统受累,早期识别并及时给予针对性的治疗利于改善患者的预后。Objective To explore the clinical characteristics,treatment and prognosis of TAFRO syndrome.Methods All patients diagnosed as Castleman disease in Peking University People′s Hospital between December 2011 and April 2019 were included.Among them,6 patients were diagnosed as TAFRO syndrome.Medical records were studied;the clinical manifestation,laboratory test,pathology,treatment and prognosis were analyzed.Recent related literatures were reviewed.Results The average age of six TAFRO syndrome patients(5 males)was 41.5 years(range,27-59 years).The patients presented as acute or subacute onset,manifested as fever,thrombocytopenia,polyserositis including pleural effusion and ascites,organomegaly,anasarca,and renal insuffciency.One patient was accompanied by hemophagocyticsyndrome,one patient was accompanied by hypothyroidism,six patients′serum IL-6 was elevated,four patients had received the test of serum VEGF and results were all elevated,six patients′HIV antibody were negative,four patients had received HHV-8 DNA test and results were all negative.For pathology,threewere plasma cell type,twowere mixed type andonewashyaline vascular type.Renal biopsies were performed in 2 patients,showing that renal thrombotic microangiopathyassociated with subacute tubulointerstitial nephritis and secondary capillary proliferative glomerulonephritis.CHOP chemotherapy wereused in 2 patients,glucocorticoid was used in 1 patient,and glucocorticoid combined with Rituximab or Tocilizumab were used in 3 patients.Among them,one patient died because of disease progression after 5 years,other five patientsare still stable.Conclusion TAFRO syndrome is a rare disease,early recognition and appropriate treatment may improvethe prognosis.

关 键 词：TAFRO综合征 CASTLEMAN病 白细胞介素6 血管内皮生长因子 

分 类 号：R593[医药卫生—内科学]