PDF(Pubmed)
Abstract：
TAFRO syndrome is an inflammatory disorder of unknown etiology characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly. Despite great advancements in research on the TAFRO syndrome in the last decade, its diagnosis and treatment are still challenging for most clinicians because of its rarity and severity. Since the initial proposal of the TAFRO syndrome as a distinct disease entity in 2010, two independent diagnostic criteria have been developed. Although these are different in the concept of whether TAFRO syndrome is a subtype of idiopathic multicentric Castleman disease or not, they are similar except for the magnitude of lymph node histopathology. Because there have been no specific biomarkers, numerous diseases must be ruled out before the diagnosis of TAFRO syndrome is made. The standard of care has not been fully established, but interleukin-6 blockade therapy with siltuximab or tocilizumab and anti-inflammatory therapy with high-dose corticosteroids are the most commonly applied for the treatment of TAFRO syndrome. The other immune suppressive agents or combination cytotoxic chemotherapies are considered for patients who do not respond to the initial treatment. Whereas glowing awareness of this disease improves the clinical outcomes of patients with TAFRO syndrome, further worldwide collaborations are warranted.
摘要：
TAFRO综合征是一种病因不明的炎症性疾病，以血小板减少为特征，Anasarca,发烧,网状蛋白纤维化，肾功能不全,和器官肿大。尽管在过去十年中，TAFRO综合征的研究取得了很大进展，由于其稀有性和严重性,对大多数临床医生来说,其诊断和治疗仍然具有挑战性.自2010年首次提出TAFRO综合征作为一种独特的疾病实体以来，已经制定了两个独立的诊断标准。尽管这些在TAFRO综合征是否是特发性多中心Castleman病的亚型的概念上有所不同，除了淋巴结组织病理学的大小，它们是相似的。因为没有特定的生物标志物，在诊断TAFRO综合征之前，必须排除许多疾病。护理标准尚未完全确立,但西妥昔单抗或托珠单抗的白介素-6阻断治疗和大剂量糖皮质激素的抗炎治疗是TAFRO综合征最常用的治疗方法.其他免疫抑制剂或联合细胞毒性化学疗法被考虑用于对初始治疗无反应的患者。尽管对这种疾病的认识可以改善TAFRO综合征患者的临床结局，进一步的全球合作是必要的。
