摘要

特發性多發性卡斯爾曼氏病 (Idiopathic Multicentric Castleman Disease, iMCD)是一種罕見且異質性的淋巴增生性疾病，屬於卡斯爾曼氏病的一種亞型，主要臨床表現包括淋巴結腫大、發燒、疲倦等症狀，且致病原因未明。iMCD 的病理機轉與免疫系統的異常反應密切相關，特別是白細胞介素-6 (interleukin-6, IL-6) 的過度分泌。傳統的治療方式為使用類固醇或化學治療來控制病人相關症狀，但長期治療及預後效果不佳且有明顯副作用。Siltuximab 是一種針對IL-6 的單株抗體，能有效減少其活性，衛生福利部於2015 年核准用於治療人類免疫不全病毒 (human immunodeficiency viruses) 陰性及人類疱疹病毒第 8 型 (human herpesvirus-8) 陰性的iMCD 病人，並於2024 年2 月通過健保給付。Siltuximab 的建議劑量以體重計算，每kg 給予11 mg，每3 週為一次療程，以靜脈輸注的方式給藥。Siltuximab 在多項研究中顯示其可有效控制病情，延長無疾病進展存活期，臨床試驗數據顯示接受siltuximab 的病人中，有34% 達到腫瘤和持久的症狀緩解維持至少18 週以上。常見副作用包括上呼吸道感染 (36%)、疲勞 (34%)、皮疹 (34%) 等，具有良好的安全性與耐受性。儘管如此，siltuximab 高昂的治療成本需列入治療計畫考量因素之一，雖已納入健保給付，然而醫療資源的有效分配仍需考慮成本效益。隨著日後更多研究，我們希望iMCD 的治療策略更明確，以改善病人的長期預後和生活品質。

 

ABSTRACT

Idiopathic Multicentric Castleman Disease (iMCD) is a rare and heterogeneous lymphoproliferative disorder, classified as a subtype of Castleman Disease. It is characterized by symptoms such as lymphadenopathy, fever, and fatigue, with an unknown etiology. The pathogenesis of iMCD is closely related to abnormal immune responses, particularly the excessive secretion of interleukin-6 (IL-6). Traditional treatments, including corticosteroids and chemotherapy, aim to control symptoms but often result in poor long-term outcomes and significant side effects. Siltuximab, a monoclonal antibody targeting IL-6, effectively reduces IL-6 activity and was approved by Taiwan’s Ministry of Health and Welfare in 2015 for the treatment of human immunodeficiency viruses-negative and human herpesvirus- 8-negative iMCD patients. In February 2024, it was included in the national health insurance reimbursement program. The recommended dosage of siltuximab is 11 mg/kg administered intravenously every three weeks. Siltuximab has demonstrated efficacy in multiple studies, effectively controlling disease progression and extending progression-free survival. Clinical trial data showed that 34% of patients treated with siltuximab achieved durable remission of both tumor burden and symptoms. Common adverse effects include upper respiratory tract infections (36%), fatigue (34%), and maculopapular rash (34%), indicating good safety and tolerability. Despite its effectiveness, the annual treatment cost of siltuximab in Taiwan is approximately one million NTD. While it is covered by health insurance, its high cost should be considered in treatment planning, emphasizing the need for cost-effectiveness in resource allocation. With further research, it is anticipated that iMCD treatment strategies will become more refined, improving long-term patient outcomes and quality of life.

 

 

Submitted for publication: 2024.10.17; Accepted for publication: 2025.03.18