大量腹水伴多系统受累的TAFRO综合征一例并文献复习

TAFRO syndrome with massive ascites and multiple system involvement:a case report and literatures review

目的 探讨大量腹水伴多系统受累TAFRO综合征患者的识别及临床表现。方法 选取2015年11月航天中心医院出现大量腹水并多系统损伤的患者1例,回顾性分析其入院资料及临床表现、实验室检查结果,并复习相关文献。结果 本例患者女性,39岁,急性起病,发热后出现大量腹水,伴贫血、PLT减少,SCr升高伴少尿。查体:皮肤巩膜黄染,腹膨隆,无压痛及反跳痛,移动性浊音阳性,肠鸣音弱,右下腹留置腹腔引流管,引流液呈血性,双下肢水肿。实验室结果显示,WBC 8.45×10^(9)/L,RBC 1.73×10^(12)/L,Hb 53 g/L,PLT 11×10^(9)/L,BUN 51.6 mmol/L,SCr 232.9μmol/L,免疫球蛋白G 1 730 mg/dl(正常范围:751~1 560 mg/dl),乳酸脱氢酶569.0 IU/L。诊断考虑TAFRO综合征。给予肾脏替代及支持治疗,配合免疫抑制剂,患者临床症状好转。结论 TAFRO综合征与一些自身免疫性疾病背景相同,临床表现为多系统受累,起病急,发病罕见,鉴别比较困难,预后凶险。患者需紧急医疗处理,可以获得良好的临床效果。

Objective To explore the identification and clinical manifestations of TAFRO syndrome patients with massive ascites and multiple system involvement.Methods A patient with massive ascites and multiple system injuries in Aerospace Center Hospital in November 2015 was selected,and the admission data,clinical manifestations and laboratory examination results were analyzed retrospectively,and the related literatures were reviewed.Results The patient was a 39 years old female with acute onset and accompanied by massive ascites after fever,anemia,thrombocytopenia,increased SCr and oliguria.Physical examination showed that yellow staining of the skin and sclera,abdominal distension,no tenderness and rebound pain,positive shifting dullness,weak bowel sounds,an abdominal drainage tube with bloody drainage in the lower right abdomen,and lower limb edema.Laboratory test showed that WBC 8.45×10^(9)/L,RBC 1.73×10^(12)/L,Hb 53g/L,PLT 11×10^(9)/L,BUN 51.6 mmol/L,SCr 232.9μmol/L,Immunoglobulin G 1730 mg/dl(normal range 751-1560 mg/dl),lactate dehydrogenase 569.0 IU/L.Diagnosis of TAFRO syndrome was considered.The patient was given renal replacement and supportive treatment,combined with immunosuppressants,and the patient's clinical symptoms improved.Conclusions TAFRO syndrome has the same background as some autoimmune diseases,with clinical manifestations of multiple system involvement,acute onset,rare occurrence,difficult differentiation,and dangerous prognosis.Patients need emergency medical treatment and can achieve good clinical outcomes.