Thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly (TAFRO) syndrome is a heterogeneous entity manifesting with a constellation of symptoms described above that can occur in the context of idiopathic multicentric Castleman disease (iMCD) as well as infectious diseases, malignancies, and rheumatologic disorders. So, iMCD-TAFRO is an aggressive subtype of iMCD with TAFRO syndrome and often hyper-vascularized lymph nodes. Since we proposed diagnostic criteria of iMCD-TAFRO in 2016, we have accumulated new insights on the disorder and additional cases have been reported worldwide. In this systematic review and cohort analysis, we established and validated a definition for iMCD-TAFRO. First, we searched PubMed and Japan Medical Abstracts Society databases using the keyword “TAFRO” to extract cases. Patients with possible systemic autoimmune diseases and hematologic malignancies were excluded. Our search identified 54 cases from 50 articles. We classified cases into three categories: (1) iMCD-TAFRO (TAFRO syndrome with lymph node histopathology consistent with iMCD), (2) possible iMCD-TAFRO (TAFRO syndrome with no lymph node biopsy performed and no other co-morbidities), and (3) TAFRO without iMCD or other co-morbidities (TAFRO syndrome with lymph node histopathology not consistent with iMCD or other comorbidities). Based on the findings, we propose an international definition requiring four clinical criteria (thrombocytopenia, anasarca, fever/hyperinflammatory status, organomegaly), renal dysfunction or characteristic bone marrow findings, and lymph node features consistent with iMCD. The definition was validated with an external cohort (the ACCELERATE Natural History Registry). The present international definition will facilitate a more precise and comprehensive approach to the diagnosis of iMCD-TAFRO.

中文翻译：

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经验证的特发性多中心 Castleman 病的血小板减少症、全身性水肿、发热、网状纤维化、肾功能不全和器官肿大临床亚型 (TAFRO) 的国际定义

血小板减少症、全身水肿、发热、网状纤维化、肾功能不全和器官肿大 (TAFRO) 综合征是一种异质性实体，表现为上述一系列症状，可在特发性多中心 Castleman 病 (iMCD) 以及传染病的背景下发生，恶性肿瘤和风湿病。因此，iMCD-TAFRO 是 iMCD 的一种侵袭性亚型，伴有 TAFRO 综合征，并且通常有高血管化淋巴结。自 2016 年我们提出 iMCD-TAFRO 的诊断标准以来，我们对该疾病积累了新的见解，并且在全球范围内报告了更多病例。在这项系统评价和队列分析中，我们建立并验证了 iMCD-TAFRO 的定义。首先，我们使用关键字“TAFRO”搜索 PubMed 和日本医学文摘协会数据库以提取病例。排除可能患有全身性自身免疫性疾病和血液系统恶性肿瘤的患者。我们的搜索从 50 篇文章中确定了 54 个案例。我们将病例分为三类：（1）iMCD-TAFRO（TAFRO 综合征，淋巴结组织病理学与 iMCD 一致），（2）可能的 iMCD-TAFRO（TAFRO 综合征，未进行淋巴结活检且无其他合并症），以及(3) 无 iMCD 或其他合并症的 TAFRO（TAFRO 综合征伴淋巴结组织病理学与 iMCD 或其他合并症不一致）。基于这些发现，我们提出了一个国际定义，需要四个临床标准（血小板减少、全身水肿、发热/高炎症状态、器官肿大）、肾功能不全或特征性骨髓发现以及与 iMCD 一致的淋巴结特征。该定义通过外部队列（ACCELERATE 自然历史登记处）进行了验证。目前的国际定义将有助于对 iMCD-TAFRO 进行更精确和全面的诊断。