Management of Castleman’s Disease: Recent Advances and Case Sharing
RSM Wong
Hong Kong J Radiol 2012;15(Suppl):S75-80
Castleman’s disease is an uncommon lymphoproliferative disorder characterised by hyperplasia of lymphoid tissue. There are three histopathological types of Castleman’s disease: hyaline vascular variant, plasma cell variant, and mixed cellularity. Hyaline vascular Castleman’s disease is typically unicentric in presentation, while the plasma cell variant tends to be multicentric. Infection with human herpes virus 8 and interleukin-6 production are implicated in the pathogenesis of the disease. Unicentric Castleman’s disease is often curable by surgical removal, while multicentric Castleman’s disease usually manifests with constitutional symptoms and requires systemic treatment. Cytotoxic chemotherapy and / or steroids have been widely used as treatment of multicentric Castleman’s disease, with varying degrees of response. The discovery of molecular components and mechanisms underlying the disease pathogenesis has been translated into targeted therapeutic approaches. Particularly, the use of anti-CD20 antibody, rituximab, antibodies targeting the interleukin-6 pathways, and antiviral agents have demonstrated efficacy in case reports and case series of multicentric Castleman’s disease. In this article, we report on two patients with multicentric Castleman’s disease and summarise the current understanding of the disease pathogenesis and therapeutic approaches to this complex and heterogeneous disease.
中文摘要
Castleman病的治理：最新進展與病例分享
王紹明
Castleman病是一種罕見的淋巴組織增生病變，其特徵為淋巴組織腫大。Castleman病可分為三種病理 類型：透明血管型、漿細胞型及混合型。Castleman病的透明血管型屬於局部型，而漿細胞型屬於多 發型。發病機制與人類8型泡疹病毒感染及白介素-6（IL-6）的產生有關。局部型Castleman病通常可 以手術切除治理。多發型Castleman病表現為全身症狀，需要全身性系統治療。多發型一般會使用細 胞毒性化療和／或類固醇，但病人反應有很大差異。從發現Castleman病的分子成分和機制可找出針 對性療法。文獻中的病例報告指出抗CD-20抗體、rituximab、抗IL-6抗體治療均對於多發型Castleman 病有成效。本文報告多發型Castleman病的兩名患者，並總結對這複雜和臨床表現多樣化的疾病的病 理機制及治療方法。