Wu C B, Zhang H Y, Shao S H, Dou L W, Zhou Q Y, Liu Y, Gao W B, Zhu J H
Department of Emergency Peking University People's Hospital, Beijing 100044, China.
Department of Hematology, Peking University People's Hospital, Beijing 100044, China.
Zhonghua Yi Xue Za Zhi. 2020 Mar 3;100(8):624-628. doi: 10.3760/cma.j.issn.0376-2491.2020.08.012.
To explore the clinical characteristics, treatment and prognosis of TAFRO syndrome. 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. 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. TAFRO syndrome is a rare disease, early recognition and appropriate treatment may improvethe prognosis.
探讨TAFRO综合征的临床特征、治疗及预后。纳入2011年12月至2019年4月期间在北京大学人民医院诊断为Castleman病的所有患者。其中,6例患者被诊断为TAFRO综合征。研究病历;分析临床表现、实验室检查、病理、治疗及预后。复习近期相关文献。6例TAFRO综合征患者(5例男性)的平均年龄为41.5岁(范围27 - 59岁)。患者呈急性或亚急性起病,表现为发热、血小板减少、多浆膜炎(包括胸腔积液和腹水)、器官肿大、全身水肿及肾功能不全。1例患者伴有噬血细胞综合征,1例患者伴有甲状腺功能减退,6例患者血清IL - 6升高,4例患者接受血清VEGF检测结果均升高,6例患者HIV抗体均为阴性,4例患者接受HHV - 8 DNA检测结果均为阴性。病理方面,3例为浆细胞型,2例为混合型,1例为透明血管型。2例患者进行了肾活检,显示肾血栓性微血管病伴亚急性肾小管间质性肾炎及继发性毛细血管增生性肾小球肾炎。2例患者使用CHOP化疗,1例患者使用糖皮质激素,3例患者使用糖皮质激素联合利妥昔单抗或托珠单抗。其中,1例患者在5年后因疾病进展死亡,其他5例患者仍病情稳定。TAFRO综合征是一种罕见疾病,早期识别和恰当治疗可能改善预后。