Multicentric Castleman’s disease. Symptoms, diagnostics and therapy

Czech version

Authors: Zdeněk Adam ¹; Zdeněk Řehák ²; Zuzana Adamová ³; Renata Koukalová ²; Luděk Pour ¹; Marta Krejčí ¹; Ivanna Boichuk ¹; Viera Sandecká ¹; Martin Krejčí ¹; Martin Štork ¹; Sabina Ševčíková ⁴; Zdeněk Král ¹
Authors‘ workplace: Interní hematologická a onkologická klinika LF MU a FN Brno ¹; Oddělení nukleární medicíny Masarykův onkologický ústav Brno ²; Chirurgické oddělení nemocnice Frýdek-Místek ³; Ústav patologické fyziologie LF MU, Brno ⁴
Published in: Vnitř Lék 2022; 68(1): 41-53
Category: Review Articles

Overview

Castleman disease (CD) describes a group of heterogeneous hematologic disorders with characteristic histopathological features. CD can present with unicentric (UCD) or multicentric (MCD) regions of lymph node enlargement. Some cases of MCD are caused by human herpesvirus-8 (HHV-8), whereas others are HHV-8–negative/idiopathic (iMCD). Treatment of iMCD is challenging, and outcomes can be poor. In this paper, we briefly report about symptoms of iMCD and about the International, evidence‑based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease and International evidence based consensus treatment guidelines for idiopathic multicentric Castleman disease.

Keywords:

Castleman disease – siltuximab

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