Division of Hematology, Mayo Clinic, Rochester, MN; and.
Division of Translational Medicine and Human Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA.
Blood. 2020 Apr 16;135(16):1353-1364. doi: 10.1182/blood.2019000931.
Castleman disease (CD) describes a group of at least 4 disorders that share a spectrum of characteristic histopathological features but have a wide range of etiologies, presentations, treatments, and outcomes. CD includes unicentric CD (UCD) and multicentric CD (MCD), the latter of which is divided into idiopathic MCD (iMCD), human herpes virus-8 (HHV8)-associated MCD (HHV8-MCD), and polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS)-associated MCD (POEMS-MCD). iMCD can be further subclassified into iMCD-thrombocytopenia, ascites, reticulin fibrosis, renal dysfunction, organomegaly (iMCD-TAFRO) or iMCD-not otherwise specified (iMCD-NOS). Advances in diagnosis, classification, pathogenesis, and therapy are substantial since the original description of UCD by Benjamin Castleman in 1954. The advent of effective retroviral therapy and use of rituximab in HHV8-MCD have improved outcomes in HHV8-MCD. Anti-interleukin-6-directed therapies are highly effective in many iMCD patients, but additional therapies are required for refractory cases. Much of the recent progress has been coordinated by the Castleman Disease Collaborative Network (CDCN), and further progress will be made by continued engagement of physicians, scientists, and patients. Progress can also be facilitated by encouraging patients to self-enroll in the CDCN's ACCELERATE natural history registry (#NCT02817997; www.CDCN.org/ACCELERATE).
血管滤泡性淋巴结增生症（Castleman disease，CD）是一组至少包括 4 种疾病的疾病谱，这些疾病具有特征性的组织病理学表现，但病因、临床表现、治疗和结局差异很大。CD 包括局灶型 CD（unicentric Castleman disease，UCD）和多中心型 CD（multicentric Castleman disease，MCD），后者又分为特发性 MCD（idiopathic MCD，iMCD）、人疱疹病毒 8 型（human herpes virus-8，HHV8）相关 MCD（HHV8-MCD）、多发性神经病、器官肿大、内分泌病、单克隆浆细胞病、皮肤改变（polyneuropathy，organomegaly，endocrinopathy，monoclonal plasma cell disorder，skin changes，POEMS）相关 MCD（POEMS-MCD）。iMCD 可进一步细分为 iMCD 伴血小板减少、腹水、网状纤维纤维化、肾功能不全、器官肿大（iMCD-TAFRO）或 iMCD 不伴其他特指（iMCD-NOS）。自 1954 年 Benjamin Castleman 首次描述 UCD 以来，在诊断、分类、发病机制和治疗方面取得了实质性进展。有效的逆转录病毒治疗和 HHV8-MCD 中利妥昔单抗的应用改善了 HHV8-MCD 的预后。抗白细胞介素 6 靶向治疗对许多 iMCD 患者非常有效，但对于难治性病例还需要其他治疗方法。最近的大部分进展是由 Castleman 疾病协作网络（CDCN）协调的，进一步的进展将通过医生、科学家和患者的持续参与来实现。通过鼓励患者自行加入 CDCN 的 ACCELERATE 自然史登记研究（#NCT02817997；www.CDCN.org/ACCELERATE），也可以促进进展。
