Castleman disease (CD) describes a group of at least four 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), HHV8-associated MCD (HHV8-MCD), and POEMS-associated MCD. iMCD can be further sub-classified into iMCD-TAFRO (thrombocytopenia, ascites, reticulin fibrosis, renal dysfunction, organomegaly) or iMCD-NOS (not otherwise specified), who often have thrombocytosis and hypergammaglobulinemia. 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, the last of which can be facilitated by encouraging patients to self-enroll in the CDCN's ACCELERATE natural history registry (#NCT02817997, www.CDCN.org/ACCELERATE).

中文翻译：

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卡斯尔曼病概述

Castleman 病 (CD) 描述了一组至少四种疾病，这些疾病具有一系列特征性组织病理学特征，但具有广泛的病因、表现、治疗和结果。CD包括单中心CD（UCD）和多中心CD（MCD），后者分为特发性MCD（iMCD）、HHV8相关MCD（HHV8-MCD）和POEMS相关MCD。iMCD 可进一步细分为 iMCD-TAFRO（血小板减少症、腹水、网状纤维化、肾功能障碍、器官肿大）或 iMCD-NOS（未另作说明），通常有血小板增多症和高丙种球蛋白血症。自 1954 年 Benjamin Castleman 最初描述 UCD 以来，诊断、分类、发病机制和治疗取得了实质性进展。有效逆转录病毒疗法的出现和在 HHV8-MCD 中使用利妥昔单抗改善了 HHV8-MCD 的结果。抗白细胞介素 6 定向治疗对许多 iMCD 患者非常有效，但对于难治性病例需要额外的治疗。最近的大部分进展都由 Castleman 疾病协作网络 (CDCN) 协调，医生、科学家和患者的持续参与将取得进一步进展，鼓励患者自行注册可以促进最后的进展CDCN 的 ACCELERATE 自然历史登记处（#NCT02817997，www.CDCN.org/ACCELERATE）。