Hiramatsu Sumie, Ohmura Koichiro, Tsuji Hideaki, Kawabata Hiroshi, Kitano Toshiyuki, Sogabe Ayuko, Hashimoto Motomu, Murakami Kosaku, Imura Yoshitaka, Yukawa Naoichiro, Yoshifuji Hajime, Fujii Takao, Takaori-Kondo Akifumi, Mimori Tsuneyo
Department of Rheumatology and Clinical Immunology.
Nihon Rinsho Meneki Gakkai Kaishi. 2016;39(1):64-71. doi: 10.2177/jsci.39.64.
TAFRO syndrome is a newly defined disease entity which is characterized by thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly. A histological pattern of multiple lymphadenopathy of atypical Castleman's disease (CD) is also an important characteristic. A 48-year-old man was referred to our hospital with fever, asthenia, bilateral pleural effusion, ascites, generalized edema, dyspnea, hypoalbuminemia, severe thrombocytopenia, anemia, renal failure and proteinuria, whereas bacterial culture and serological and PCR tests for various viruses were all negative. A CT scan showed multiple lymphadenopathy and tissue sampling of inguinal lymph nodes showed a compatible histology with plasma cell type CD. A diagnosis of TAFRO syndrome was made. Ten days after hospitalization, sudden cardiac insufficiency and anuria developed. Despite glucocorticoid pulse therapy, tocilizumab and plasmapheresis, clinical and laboratory features did not improve. On the 34(th) hospital day, we started rituximab. His general condition started to improve in several days, and by one month later anasarca had improved drastically. Thrombocytopenia and renal function gradually improved and finally normalized. Cardiac motion also improved. This is the first report of a TAFRO syndrome patient with cardiomyopathy, who was successfully treated with rituximab.
TAFRO综合征是一种新定义的疾病实体，其特征为血小板减少、全身性水肿、骨髓纤维化、肾功能不全和器官肿大。非典型Castleman病（CD）的多发性淋巴结病组织学模式也是一个重要特征。一名48岁男性因发热、乏力、双侧胸腔积液、腹水、全身性水肿、呼吸困难、低白蛋白血症、严重血小板减少、贫血、肾衰竭和蛋白尿被转诊至我院，而各种病毒的细菌培养、血清学和PCR检测均为阴性。CT扫描显示多发性淋巴结病，腹股沟淋巴结组织活检显示与浆细胞型CD相符的组织学特征。诊断为TAFRO综合征。住院10天后，突发心功能不全和无尿。尽管进行了糖皮质激素冲击治疗、托珠单抗治疗和血浆置换，但临床和实验室指标均未改善。在住院第34天，我们开始使用利妥昔单抗。几天后他的一般状况开始改善，到一个月后全身性水肿已大幅改善。血小板减少和肾功能逐渐改善并最终恢复正常。心脏功能也有所改善。这是首例患有心肌病的TAFRO综合征患者成功接受利妥昔单抗治疗的报告。
