Chan Kah-Lok, Lade Stephen, Prince H Miles, Harrison Simon J
Department of Haematology.
Department of Anatomical Pathology, Peter MacCallum Cancer Centre.
J Blood Med. 2016 Aug 3;7:145-58. doi: 10.2147/JBM.S60514. eCollection 2016.
First described 60 years ago, Castleman disease comprises a rare and heterogeneous cluster of disorders, characterized by lymphadenopathy with unique histological features and associated with cytokine-driven constitutional symptoms and biochemical disturbances. Although unicentric Castleman disease is curable with complete surgical excision, its multicentric counterpart is a considerable therapeutic challenge. The recent development of biological agents, particularly monoclonal antibodies to interleukin-6 and its receptor, allow for more targeted disease-specific intervention that promises improved response rates and more durable disease control; however, further work is required to fill knowledge gaps in terms of underlying pathophysiology and to facilitate alternative treatment options for refractory cases.
Castleman病于60年前首次被描述，它是一组罕见且异质性的疾病，其特征为具有独特组织学特征的淋巴结病，并伴有细胞因子驱动的全身症状和生化紊乱。虽然单中心Castleman病通过完全手术切除可治愈，但其多中心型却是一个相当大的治疗挑战。生物制剂的最新发展，特别是针对白细胞介素-6及其受体的单克隆抗体，使得更有针对性的疾病特异性干预成为可能，有望提高缓解率并实现更持久的疾病控制；然而，在潜在病理生理学方面仍需进一步研究以填补知识空白，并为难治性病例提供更多替代治疗选择。
