Matsuhisa Takaharu, Takahashi Noriyuki, Nakaguro Masato, Sato Motoki, Inoue Eri, Teshigawara Shiho, Ozawa Yukihiro, Kondo Takeshi, Nakamura Shigeo, Sato Juichi, Ban Nobutaro
Department of General Medicine/Family & Community Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Department of Education for Community-Oriented Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Nagoya J Med Sci. 2019 Aug;81(3):519-528. doi: 10.18999/nagjms.81.3.519.
TAFRO syndrome is a novel disease concept characterized by Thrombocytopenia, Anasarca, myeloFibrosis, Renal dysfunction, Organomegaly, multiple lymphadenopathy and a histopathological pattern of atypical Castleman's disease. A 58-year-old man was diagnosed as TAFRO syndrome by clinical and histopathological findings. After receiving intensive immunosuppressive therapy, his thrombocytopenia and anasarca had not improved. He developed complications such as methicillin-resistant , gastrointestinal bleeding, peritonitis caused by , gastrointestinal perforation, and disseminated candidiasis resulting in death. Autopsy revealed disseminated candidiasis and hemophagocytic lymphohistiocytosis, with no evidence of TAFRO syndrome. During treatment, we regarded his lasting thrombocytopenia and anasarca as insufficient control of TAFRO syndrome. However, the autopsy revealed that thrombocytopenia was caused by secondary hemophagocytic lymphohistiocytosis caused by over-immunosuppression. We reviewed the published literature to identify indicators of adequate treatment, which suggested improvement of platelet count and anasarca several weeks after initial therapy. This indicated that we could not depend on the platelet count and anasarca in acute medical care after initial treatment. We should treat TAFRO syndrome based on patients' clinical status and obviate the risk of treatment-related complications caused by over-immunosuppression.
TAFRO综合征是一种以血小板减少、全身性水肿、骨髓纤维化、肾功能不全、器官肿大、多处淋巴结病以及非典型Castleman病的组织病理学模式为特征的新型疾病概念。一名58岁男性通过临床和组织病理学检查结果被诊断为TAFRO综合征。在接受强化免疫抑制治疗后，他的血小板减少和全身性水肿并未改善。他出现了诸如耐甲氧西林菌感染、胃肠道出血、由[未明确病原体]引起的腹膜炎、胃肠道穿孔以及播散性念珠菌病等并发症，最终导致死亡。尸检显示有播散性念珠菌病和噬血细胞性淋巴组织细胞增生症，未发现TAFRO综合征迹象。在治疗过程中，我们将他持续的血小板减少和全身性水肿视为TAFRO综合征控制不佳。然而，尸检显示血小板减少是由过度免疫抑制导致的继发性噬血细胞性淋巴组织细胞增生症引起的。我们查阅了已发表的文献以确定充分治疗的指标，文献表明初始治疗数周后血小板计数和全身性水肿会有所改善。这表明在初始治疗后的急性医疗护理中，我们不能仅依赖血小板计数和全身性水肿情况。我们应根据患者的临床状况来治疗TAFRO综合征，避免过度免疫抑制引起的治疗相关并发症风险。
