PDF(Pubmed)
Abstract：
TAFRO syndrome is defined by the presence of thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis/renal dysfunction (R), and organomegaly (O) and can be seen with idiopathic multicentric Castleman disease (iMCD) or as an isolated process without iMCD. Although the diagnosis of iMCD in patients with TAFRO can be challenging to make, iMCD should remain high on the differential diagnosis. Similar to iMCD, the pathophysiology of TAFRO is not well understood but is thought to be related to hypercytokinemia, with interleukin (IL)-6 playing a pivotal role. Anti-IL-6 monoclonal antibody therapy is an effective treatment modality for iMCD, but to date, there is no clear guidance on treatment of TAFRO in the absence of definitive diagnosis of iMCD, leading to suboptimal management and high morbidity. We report a case of TAFRO syndrome and demonstrate benefit with the empiric use of anti-IL-6 antibody therapy in the context of delayed diagnosis of iMCD.
摘要：
TAFRO综合征的定义是血小板减少症（T）的存在，anasarca(A),发烧（F），网织蛋白纤维化/肾功能障碍（R），和器官肿大（O），可以在特发性多中心Castleman病（iMCD）或无iMCD的孤立过程中看到。尽管在TAFRO患者中诊断iMCD可能具有挑战性，iMCD在鉴别诊断中应保持较高。类似于iMCD,TAFRO的病理生理学尚不清楚，但被认为与高细胞因子血症有关，白细胞介素（IL）-6发挥关键作用。抗IL-6单克隆抗体治疗是iMCD的有效治疗方式,但迄今为止,在没有明确诊断iMCD的情况下，对TAFRO的治疗没有明确的指导，导致管理欠佳和发病率高。我们报告了一例TAFRO综合征，并证明了在iMCD诊断延迟的情况下经验性使用抗IL-6抗体治疗的益处。
