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The clinical, laboratory, and radiologic improvement due to siltuximab treatment in idiopathic multicentric Castleman’s disease

Korean Journal of Internal Medicine 2021년 36권 2호 p.424 ~ 432
민기준, 전영우, 박성수, Park Silvia, 신승환, Yahng Seung-Ah, 윤재호, 이승은, 조병식, 엄기성, 김유진, 이석, 김희제, 민창기, 김동욱, 이종욱, 조석구,
소속 상세정보
민기준 ( Min Gi-June ) - Catholic University College of Medicine Seoul St. Mary’s Hospital Department of Hematology
전영우 ( Jeon Young-Woo ) - Catholic University College of Medicine Seoul St. Mary’s Hospital Department of Hematology
박성수 ( Park Sung-Soo ) - Catholic University College of Medicine Seoul St. Mary’s Hospital Department of Hematology
 ( Park Silvia ) - Catholic University College of Medicine Seoul St. Mary’s Hospital Department of Hematology
신승환 ( Shin Seung-Hawn ) - Catholic University College of Medicine Yeouido St. Mary’s Hospital Department of Hematology
 ( Yahng Seung-Ah ) - Catholic University College of Medicine Incheon St Mary’s Hospital Department of Hematology
윤재호 ( Yoon Jae-Ho ) - Catholic University College of Medicine Seoul St. Mary’s Hospital Department of Hematology
이승은 ( Lee Sung-Eun ) - Catholic University College of Medicine Seoul St. Mary’s Hospital Department of Hematology
조병식 ( Cho Byung-Sik ) - Catholic University College of Medicine Seoul St. Mary’s Hospital Department of Hematology
엄기성 ( Eom Ki-Seong ) - Catholic University College of Medicine Seoul St. Mary’s Hospital Department of Hematology
김유진 ( Kim Yoo-Jin ) - Catholic University College of Medicine Seoul St. Mary’s Hospital Department of Hematology
이석 ( Lee Seok ) - Catholic University College of Medicine Seoul St. Mary’s Hospital Department of Hematology
김희제 ( Kim Hee-Je ) - Catholic University College of Medicine Seoul St. Mary’s Hospital Department of Hematology
민창기 ( Min Chang-Ki ) - Catholic University College of Medicine Seoul St. Mary’s Hospital Department of Hematology
김동욱 ( Kim Dong-Wook ) - Catholic University College of Medicine Seoul St. Mary’s Hospital Department of Hematology
이종욱 ( Lee Jong-Wook ) - Catholic University College of Medicine Seoul St. Mary’s Hospital Department of Hematology
조석구 ( Cho Seok-Goo ) - Catholic University College of Medicine Seoul St. Mary’s Hospital Department of Hematology

Abstract


Background/Aims: Idiopathic multicentric Castleman disease (iMCD) comprises approximately 30% of all cases of Castleman disease. It is characterized by constitutional symptoms, enlarged lymph nodes at multiple anatomical sites, and laboratory test abnormalities, which are primarily related to the overproduction of interleukin 6 (IL-6). Siltuximab is a human-mouse chimeric immunoglobulin G1κ monoclonal antibody against human IL-6. In view of the limited treatment options for iMCD, this study aimed to evaluate the efficacy and safety of siltuximab in the management of this condition.

Methods: In this real-world retrospective study, we administered siltuximab to 15 patients with iMCD who previously received conventional chemotherapy and/or steroid pulse therapy. The median time to a durable symptomatic response was 22 days (range, 17 to 56). The serum hemoglobin and albumin levels and erythrocyte sedimentation rates significantly normalized after the first 3 months of siltuximab treatment. Lymph node involution, assessed using imaging, was relatively gradual, demonstrating a complete or partial response at 6 months.

Results: On an average, the improvements in clinical, laboratory, and radiologic parameters of iMCD in responders were observed after one, three, and eight cycles of siltuximab treatment, respectively. Siltuximab demonstrated a favorable safety profile, and prolonged treatment was well-tolerated.

Conclusions: Despite the small sample size of the present study, the results are encouraging and demonstrate the potential of siltuximab as the first-line treatment of iMCD. Further large multicenter studies are needed to evaluate the clinical outcomes and adverse events associated with siltuximab.

키워드

Multi-centric Castleman's disease; Siltuximab; Interleukin-6; Tolerance

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