Vnitr Lek 2021, 67(8):465-473 | DOI: 10.36290/vnl.2021.121

Unicentric Castleman's disease. Symptoms, diagnostics and therapy

Zdeněk Adam¹, Zdeněk Řehák², Zuzana Adamová³, Renata Koukalová², Luděk Pour¹, Marta Krejčí¹, Ivanna Boichuk¹, Martin Krejčí¹, Martin Štork¹, Sabina Ševčíková⁴, Zdeněk Král¹

¹ Interní hematologická a onkologická klinika LF MU a FN Brno

² Oddělení nukleární medicíny, Masarykův onkologický ústav Brno

³ Chirurgické oddělení nemocnice Vsetín

⁴ Ústav patologické fyziologie LF MU Brno

Castleman disease (CD) includes a group of rare and heterogeneous disorders with characteristic lymph node histopathological abnormalities. CD can occur in a single lymph node station, which is referred to as unicentric CD (UCD). CD can also involve multicentric lymphadenopathy and inflammatory symptoms - multicentric Castleman disease. The first-ever diagnostic and treatment guidelines were recently developed for UCD and published 2020. Complete surgical resection is often curative and is therefore the preferred first-line therapy, if possible. The management of unresectable UCD is more challenging. Existing evidence supports that asymptomatic unresectable UCD may be observed. The anti-interleukin-6 monoclonal antibody siltuximab should be considered for unresectable UCD patients with an inflammatory syndrome. Unresectable UCD that is symptomatic because of compression of vital neighbouring structures may be rendered amenable to resection by medical therapy (rituximab, steroids), radiotherapy, or embolization. In this article, we report about the symptoms of this disease and about the diagnostics recommendation published in the International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease and about the therapeutic recommendation published in International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease published in the year 2020.

Keywords: Castleman's disease, siltuximab, rituximab.

Published: December 9, 2021  Show citation