Treatment of the multicentric and unicentric forms of Castleman disease

Czech version

Authors: Z. Král ¹; Z. Adam ¹; M. Krejčí ¹; L. Pour ¹; P. Volfová ²; M. Ježová ³; Z. Řehák ⁴; R. Koukalová ⁴
Authors‘ workplace: Interní hematologická a onkologická klinika LF MU a FN Brno ¹; Interní hematologická a onkologická klinika – Centrum molekulární biologie a genové terapie, sekce oportunních, infekcí, Dětská nemocnice ²; Ústav patologie LF MU a FN Brno ³; Oddělení nukleární medicíny, Centrum PET, Masarykův onkologický ústav ⁴
Published in: Transfuze Hematol. dnes,26, 2020, No. 3, p. 186-195.
Category: Review/Educational Papers

Overview

Castleman disease is a very rare illness. The multicentric form most often presents as a systemic inflammatory reaction, while the unicentric form involves local symptoms induced by a localised mass. Siltuximab (an interleukin 6 antibody) is registered in Europe for the treatment of the multicentric form. Tocilizumab, an antibody against the interleukin receptor, is also used outside Europe. B-lymphocytes are involved in the pathophysiology of this disease, which is why the anit-CD20 antibody is also highly effective and its use is rising in the Czech Republic. Other drugs with efficacy confirmed in case reports include thalidomide, lenalidomide and anakinra. TAFRO syndrome, a rare variant of Castleman disease that may lead to anasarca, has been successfully treated with ciclosporine. Chemotherapy based regimens used in lymphomas may be considered in cases with a highly aggressive course (sepsis like).

Keywords:

Castleman disease – TAFRO syndrome – siltuximab – rituximab – anakinra – cyclosporine

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