TAFRO综合征临床分析

Clinical Anslysis of TAFRO Syndrome

目的:探讨1例TAFRO综合征患者的临床特点及诊疗方案,加强对该类型罕见病的认识。方法:回顾性分析甘肃省人民医院收治的1例TAFRO综合征患者的临床表现、诊疗过程及预后。结果:结合实验室检查、骨髓学检查、影像学检查及病理学等检查,患者诊断为TAFRO综合征,给予泊马度胺(2-3 mg/d,d 1-21)、环磷酰胺(300 mg/m2,0.54 g每周一次)、地塞米松(20 mg/d,每周两天)方案治疗,3个周期后患者血小板计数、血肌酐、降钙素原等指标均恢复正常,全身水肿消褪,评估病情缓解,治疗效果好。结论:TAFRO综合征罕见,多系统受累,进展快,预后差,“泊马度胺+环磷酰胺+地塞米松”方案治疗有助于改善TAFRO综合征患者的生存预后。

Objective:To investigate the clinical characteristics,diagnosis,and treatment of one patient with TAFRO syndrome,and to strengthen the understanding of this rare type.Methods:The clinical manifestations,diagnosis and treatment process,and prognosis of the patient admitted in Gansu Provincial People′ s Hospital were retrospectively analyzed.Results:Combined with laboratory tests,bone marrow examination,imaging,pathology,etc,the patient was diagnosed with TAFRO syndrome.After three cycles of treatment with pomalidomide(2-3mg/d,d1-21),cyclophosphamide(300mg/m2,0.54g once a week)and dexamethasone(20mg/d,two days a week),platelet count,serum creatinine and procalcitonin returned to normal,the systemic edema disappeared,and the patient′ s condition was alleviated.The therapeutic effect was good.Conclusion:TAFRO syndrome is rare,involves multiple systems,progresses rapidly,and has a worse prognosis.The choice of the“Pomalidomide+cyclophosphamide+dexamethasone”regimen is help to improve the survival prognosis of patient with TAFRO syndrome.