Castleman disease.
Castleman disease.
复制标题
DOI：
10.1038/s41572-021-00317-7
复制
发表时间：
2021-11-25
期刊：
影响因子：
--
通讯作者：
中科院分区：
文献类型：
--
作者：
Castleman病（CD）是一组具有共同形态学特征的异质性疾病，根据临床表现和病程可分为单中心CD和多中心CD（MCD）。单中心CD涉及孤立性淋巴结肿大和轻度症状，切除手术往往是治愈的。MCD包括与卡波西肉瘤疱疹病毒（KSHV）（也称为人疱疹病毒8）相关的一种形式和KSHV阴性特发性形式（iMCD）。iMCD可与重度综合征相关，如TAFRO（血小板减少症、腹水、发热、网硬蛋白纤维化和器官肿大）或POEMS（多发性神经病、器官肿大、内分泌病、单克隆浆细胞疾病和皮肤变化）。KSHV-MCD通常发生在HIV感染或其他免疫缺陷的情况下。KSHV和HIV之间的相互作用增加了发生KSHV诱导疾病的风险，包括KSHV-MCD、KSHV淋巴细胞增殖、KSHV炎性细胞因子综合征、原发性渗出性淋巴瘤和卡波西肉瘤。CD诊断需要多方面的方法，包括临床表现和影像学，病理学特征和分子病毒学。B细胞定向单克隆抗体治疗是KSHV-MCD的标准治疗，抗IL-6治疗是推荐的一线治疗，也是美国FDA和EMA批准的唯一iMCD治疗。
Castleman disease (CD), a heterogeneous group of disorders that share morphological features, is divided into unicentric CD and multicentric CD (MCD) according to the clinical presentation and disease course. Unicentric CD involves a solitary enlarged lymph node and mild symptoms and excision surgery is often curative. MCD includes a form associated with Kaposi sarcoma herpesvirus (KSHV) (also known as human herpesvirus 8) and a KSHV-negative idiopathic form (iMCD). iMCD can present in association with severe syndromes such as TAFRO (thrombocytopenia, ascites, fever, reticulin fibrosis and organomegaly) or POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder and skin changes). KSHV-MCD often occurs in the setting of HIV infection or another cause of immune deficiency. The interplay between KSHV and HIV elevates the risk for the development of KSHV-induced disorders, including KSHV-MCD, KSHV-lymphoproliferation, KSHV inflammatory cytokine syndrome, primary effusion lymphoma and Kaposi sarcoma. A CD diagnosis requires a multidimensional approach, including clinical presentation and imaging, pathological features, and molecular virology. B cell-directed monoclonal antibody therapy is the standard of care in KSHV-MCD, and anti-IL-6 therapy is the recommended first-line therapy and only treatment of iMCD approved by the US FDA and EMA.
登录
查看更多内容
影响因子：
6.5
作者：
Boutboul, David;Fadlallah, Jehane;Galicier, Lionel
通讯作者：
Galicier, Lionel
影响因子：
20.3
作者：
Casper, C;Nichols, WG;Wald, A
通讯作者：
Wald, A
影响因子：
3.3
作者：
Brousset, P;Cesarman, E;Delsol, G
通讯作者：
Delsol, G
DOI：
10.1073/pnas.1600587113
发表时间：
2016-07-12
影响因子：
11.1
作者：
Bhatt, Aadra Prashant;Wong, Jason P.;Damania, Blossom
通讯作者：
Damania, Blossom
影响因子：
20.3
作者：
Bélec, L;Mohamed, AS;Gherardi, RK
通讯作者：
Gherardi, RK