Efficacy and safety of regimens used for the treatment of multicentric Castleman disease: A systematic review. (13th July 2022)

• Record Type: Journal Article
• Authors: Rehman, Mohammad Ebad Ur; Chattaraj, Asmi; Neupane, Karun; Rafae, Abdul; Saeed, Sajeel; Basit, Jawad; Ibrahim, Atif; Khouri, Jack; Mukherjee, Sudipto; Anwer, Faiz
• Is Part Of: European journal of haematology. Volume 109:Number 4(2022)
• Page Start: 309
• Page End: 320
• Publication Date: 2022-07-13
• Subjects: Castleman disease -- HHV8 -- IL‐6 -- lymphadenopathy -- rituximab -- siltuximab; Hematology -- Periodicals; Blood -- Diseases -- Periodicals; Blood -- Periodicals; 616.15005
• DOI: 10.1111/ejh.13823
• Languages: English
• ISSNs: 0902-4441

Abstract

Abstract: Objectives: Treatment options for multicentric Castleman disease (MCD) remain limited. The only FDA‐approved drug is siltuximab for idiopathic MCD (iMCD), but the response rate with siltuximab is less than 50%. We performed a systematic review to examine the efficacy and safety of various regimens used for the treatment of MCD. Methods: A database search on PubMed, Embase, Cochrane, Web of Science, and Clinicaltrials.gov using the terms "Castleman disease, " "treatment outcome, " and "patient safety" was done. Results and Conclusions: Results from a randomized controlled trial and an extension study highlighted the efficacy and long‐term safety of siltuximab for iMCD; other trials showed tocilizumab to be a suitable alternative. A recent trial reported high response rates with thalidomide in iMCD patients. Promising results were reported for bortezomib in relapsed/ refractory MCD. For human herpesvirus‐8 (HHV8)‐associated MCD, rituximab along with doxorubicin therapy followed by maintenance with zidovudine and valganciclovir is the most effective therapy. A single‐arm trial has highlighted the potential role of tocilizumab in HHV8‐MCD. Data for these regimens are limited and mostly comprise nonrandomized trials. Further research on emerging agents could have a major impact on the treatment of this rare disease.