Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine, Kagawa University, Kagawa, Japan.
Mod Rheumatol Case Rep. 2021 Jan;5(1):145-151. doi: 10.1080/24725625.2020.1789304. Epub 2020 Jul 15.
Recently, a unique clinicopathologic variant of multicentric Castleman disease, TAFRO (i.e. thrombocytopenia, anasarca, fever, renal failure or reticulin fibrosis and organomegaly) syndrome, has been identified in Japan. Previous reports have shown that affected patients usually respond to anti-interleukin 6 (IL-6) receptor antibody, but not all patients achieve remission. Here, we present a 62-year-old man meeting the criteria of TAFRO syndrome. Serum, plasma and ascites levels of cytokines, including IL-6 and vascular endothelial growth factor, were markedly elevated. Tocilizumab, an anti-IL-6 receptor antibody, and corticosteroids were initially used to treat the increase in acute inflammatory proteins and the anasarca, resulting in decreased cytokine levels. However, the patient showed a rapidly progressive course of anasarca and ascites, and an increase in acute inflammatory proteins and cytokine levels shortly thereafter. Rituximab, an anti-CD20 antibody, successfully induced remission of disease symptoms and decreased cytokine levels. The patient was successfully treated with rituximab despite being refractory to tocilizumab and corticosteroids. During our patient's clinical course, monitoring cytokine profiles, especially vascular endothelial growth factor, was useful in tracking the disease activity of TAFRO syndrome.
最近，在日本发现了一种独特的多中心 Castleman 病的临床病理变异型，TAFRO（即血小板减少、浮肿、发热、肾衰竭或网状纤维和器官肿大）综合征。先前的报告表明，受影响的患者通常对白细胞介素 6（IL-6）受体抗体有反应，但并非所有患者都能缓解。在这里，我们介绍了一位符合 TAFRO 综合征标准的 62 岁男性。血清、血浆和腹水细胞因子水平，包括白细胞介素 6 和血管内皮生长因子，明显升高。托珠单抗，一种抗白细胞介素 6 受体抗体，和皮质类固醇最初用于治疗急性炎症蛋白和浮肿的增加，导致细胞因子水平降低。然而，患者随后出现了迅速进展的浮肿和腹水、急性炎症蛋白和细胞因子水平的增加。利妥昔单抗，一种抗 CD20 抗体，成功地诱导了疾病症状的缓解，并降低了细胞因子水平。尽管对托珠单抗和皮质类固醇耐药，利妥昔单抗仍成功地治疗了该患者。在我们患者的临床过程中，监测细胞因子谱，特别是血管内皮生长因子，有助于跟踪 TAFRO 综合征的疾病活动。
