特发性多中心Castleman病(iMCD)和TAFRO综合征存在多种症状,被认为是由过度的炎症细胞因子和趋化因子引起的,但是潜在的机制是未知的。iMCD大致分为两种类型:iMCD-NOS和iMCD-TAFRO,有不同的实验室发现,病理特征,以及对治疗的反应。人们认为iMCD-NOS,特别是IPL类型,由于其IL-6中心的概况,对IL-6抑制剂有良好的反应。iMCD-TAFRO经常进展迅速和认真,类似于TAFRO综合征。细胞因子水平升高,包括IL-1β,TNF-α,IL-10和IL-23,以及趋化因子如CXCL13和CXCL-10(尤其是在iMCD-TAFRO中),SAA,和VEGF,与疾病的病理有关。最近的研究已经确定了关键的信号通路,包括PI3K/Akt/mTOR和JAK-STAT3,以及由I型IFN调节的信号通路,在iMCD-TAFRO中至关重要。这些结果表明,优势途径可能在亚型之间有所不同。需要进一步研究外周血和淋巴结以确定iMCD-NOS/iMCD-TAFRO/TAFRO综合征的疾病谱。
Idiopathic multicentric Castleman disease (iMCD) and TAFRO syndrome present a variety of symptoms thought to be caused by excessive inflammatory cytokines and chemokines, but the underlying mechanisms are unknown. iMCD is broadly classified into two types: iMCD-NOS and iMCD-TAFRO, which have distinct laboratory findings, pathological features, and responses to treatments. It is thought that iMCD-NOS, particularly the IPL type, responds favorably to IL-6 inhibitors due to its IL-6-centric profile. iMCD-TAFRO frequently progresses acutely and seriously, similar to TAFRO syndrome. Elevated levels of cytokines, including IL-1β, TNF-α, IL-10, and IL-23, as well as chemokines like CXCL13 and CXCL-10 (especially in iMCD-TAFRO), SAA, and VEGF, have been linked to the disease\'s pathology. Recent research has identified key signaling pathways including PI3K/Akt/mTOR and JAK-STAT3, as well as those regulated by type I IFN, as crucial in iMCD-TAFRO. These results suggest that dominant pathways may vary between subtypes. Further research into the peripheral blood and lymph nodes is required to determine the disease spectrum of iMCD-NOS/iMCD-TAFRO/TAFRO syndrome.