Onkologie. 2013:7(3):144-147

Treatment options for multicentric Castleman disease

Petr Szturz

Interní hematologická a onkologická klinika LF MU a FN Brno

Backgrounds: Castleman disease is a rare nonclonal lymphoproliferative disorder with 2 clinical (unicentric and multicentric) and

4 histomorphologic (hyaline-vascular, plasma-cell, mixed and plasmablastic) types. Typically, multicentric plasma-cell Castleman disease

is an aggressive, relapsing disorder with generalized lymphadenopathy and systemic symptoms. Due to absence of randomised comparison

studies, treatment recommendations for this disease form are not united and mostly, the practicing physicians take guidance

from retrospective reports of single cases and small groups of patients.

Aim: This paper summarizes the available treatment modalities for multicentric Castleman disease and based on an analysis of literature

sources it provides current recommendations for treating the disease.

Conclusions: The pillars for treating multicentric Castleman disease should comprise preparates from the groups of monoclonal antibodies

(tocilizumab, rituximab) and immunomodulatory drugs (thalidomide, lenalidomide). Positive aspects of thalidomide include favourable

financial calculation, the possiblity of long-term administration with lasting therapy effects and reported efficacy in rituximab-resistant cases.

Keywords: Castleman disease, tocilizumab, rituximab, immunomodulatory drugs, thalidomide, lenalidomide

Published: July 1, 2013  Show citation