Rehman · European journal of haematology 2022 · systematic review · n=?

Efficacy and safety of regimens used for the treatment of multicentric Castleman disease: A systematic review.

Cited 18 times in the scientific literature.

Level 2 - randomized trial

Systematic review including both randomized and non-randomized studies

PubMed 35770616 · doi:10.1111/ejh.13823 · record verified 2026-08-26

What was done

Authors conducted a systematic review searching PubMed, Embase, Cochrane, Web of Science, and Clinicaltrials.gov with terms related to Castleman disease, treatment outcome, and patient safety to evaluate efficacy and safety of regimens for multicentric Castleman disease (MCD).

What was found

The abstract reports qualitative narrative summaries without providing specific effect sizes, sample sizes, or response percentages. For idiopathic MCD (iMCD), siltuximab showed efficacy and long-term safety in a randomized trial and extension study, tocilizumab served as an alternative, thalidomide yielded high response rates, and bortezomib showed activity in relapsed/refractory cases. For HHV8-associated MCD, rituximab with doxorubicin followed by zidovudine and valganciclovir maintenance was identified as most effective, with tocilizumab evaluated in a single-arm trial.

Why it matters

Multicentric Castleman disease is rare with limited approved therapies and low response rates to standard treatments, making synthesized evidence on alternative targeted and cytotoxic regimens clinically relevant.

Limits

The abstract omits quantitative outcome data, total participant numbers, and study counts. The underlying evidence base is primarily constrained to non-randomized, single-arm studies.

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