Recommended by the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) as the preferred treatment for iMCD1
Regardless of disease severity or histopathology, siltuximab is recommended by the NCCN Guidelines as the preferred first-line treatment option in patients with idiopathic multicentric Castleman disease (iMCD).
NCCN Guidelines for B-Cell Lymphomas (Version 5.2022) includes treatment recommendations for iMCD
Note: All recommendations are category 2A unless otherwise indicated.
Clinical Trials: NCCN believes that the best management of any patient with cancer is in a clinical trial. Participation in clinical trials is especially encouraged.
See Criteria for Active Disease (CD-A) in the NCCN Guidelines for more information
See Diagnostic Criteria for Idiopathic MCD (CD-B) in the NCCN Guidelines for more information.
Rituximab and hyaluronidase human injection for subcutaneous use may be substituted for rituximab after patients have received the first full dose of rituximab by intravenous infusion. This substitution cannot be made for rituximab used in combination with ibritumomab tiuxetan.
An FDA-approved biosimilar is an appropriate substitute for rituximab.
Response assessment using the imaging modalities performed during workup (C/A/P CT with contrast or PET-CT).
Encourage biopsy to rule out transformation to DLBCL or concomitant development of other malignancies or opportunistic infections.
Rituximab ± prednisone may repeat without limit if progression ≥6 months after completion of rituximab.
Abbreviations: C/A/P CT, chest-abdomen-pelvis computed tomography; DLBCL, diffuse large B-cell lymphoma; FDA, US Food and Drug Administration; HHV-8(-), human herpesvirus-8 negative; HIV-1(-), human immunodeficiency virus type 1 negative; iMCD, idiopathic multicentric Castleman disease; MCD, multicentric Castleman disease; PET-CT, positron emission tomography–computed tomography.
Reference: 1. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for B-Cell Lymphomas V.5.2022. © National Comprehensive Cancer Network, Inc. 2022. All rights reserved. Accessed August 15, 2022. To view the most recent and complete version of the guideline, go online to NCCN.org. NCCN makes no warranties of any kind whatsoever regarding their content, use or application and disclaims any responsibility for their application or use in any way.