Korean J Intern Med.  2021 Mar;36(2):424-432. 10.3904/kjim.2019.330.

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

Affiliations
  • 1Department of Hematology, Seoul St. Mary’s Hematology Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
  • 2Department of Hematology, Yeouido St. Mary’s Hematology Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
  • 3Department of Hematology, Incheon St. Mary’s Hematology Hospital, College of Medicine, The Catholic University of Korea, Incheon, Korea

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.

Keyword

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