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Abstract：
TAFRO syndrome is a rare and aggressive inflammatory entity characterized by thrombocytopenia, anasarca, fever, renal failure, reticulin fibrosis, and organomegaly. This entity supposes a diagnostic and therapeutic challenge due to its significant overlap with Castleman\'s disease. However, distinct clinical and histological features warrant its classification as a separate subtype of idiopathic multicentric Castleman\'s disease (iMCD). While recent modifications have been made to the diagnostic criteria for iMCD, these criteria lack specificity for this particular condition, further complicating diagnosis. Due to its inflammatory nature, several complex molecular signaling pathways are involved, including the JAK-STAT pathway, NF-kB, and signal amplifiers such as IL-6 and VEGF. Understanding the involvement of immune dysfunction, some infectious agents, genetic mutations, and specific molecular and signaling pathways could improve the knowledge and management of the condition, leading to effective treatment strategies. The current therapeutic approaches include corticosteroids, anti-IL6 drugs, rituximab, and chemotherapy, among others, but response rates vary, highlighting the need for personalized strategies. The prognosis is uncertain due to diagnostic difficulties, emphasizing the importance of early intervention and appropriate targeted treatment. This comprehensive review examines the evolving landscape of TAFRO syndrome, including the pathophysiology, diagnostic criteria, treatment strategies, prognosis, and future perspectives.
摘要：
TAFRO综合征是一种罕见且侵袭性的炎症实体，以血小板减少为特征，Anasarca,发烧,肾功能衰竭,网状蛋白纤维化，和器官肿大。由于其与Castleman病的显着重叠，该实体提出了诊断和治疗挑战。然而,不同的临床和组织学特征保证将其分类为特发性多中心Castleman病(iMCD)的独立亚型.虽然最近对iMCD的诊断标准进行了修改，这些标准缺乏这种特殊情况的特异性，进一步复杂的诊断。由于其炎症性质，涉及几个复杂的分子信号通路，包括JAK-STAT途径,NF-kB,和信号放大器如IL-6和VEGF。了解免疫功能障碍的参与，一些传染因子,基因突变,和特定的分子和信号通路可以提高知识和管理的条件，导致有效的治疗策略。目前的治疗方法包括皮质类固醇，抗IL6药物，利妥昔单抗,和化疗，其中,但是反应率各不相同，强调个性化战略的必要性。由于诊断困难，预后不确定，强调早期干预和适当针对性治疗的重要性。这篇全面的综述探讨了TAFRO综合征的演变格局，包括病理生理学，诊断标准,治疗策略,预后,和未来的前景。
