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Non-infectious and non-malignant lymphadenopathy – idiopathic (HHV-8 negative) Castleman disease


Authors: Z. Adam 1;  P. Volfová 2;  M. Krejčí 1;  R. Koukalová 3;  Z. Řehák 3;  A. Čermák 4;  L. Pour 1
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 LF MU a FN Brno 2;  Oddělení nukleární medicíny, Centrum PET, Masarykův onkologický ústav 3;  Klinika urologie LF MU a FN Brno 4
Published in: Transfuze Hematol. dnes,24, 2018, No. 3, p. 152-165.
Category:

Overview

First described 60 years ago, Castleman disease consists of a rare and heterogeneous group of disorders, characterized by lymphadenopathy with unique histological features and associated with cytokine-driven constitutional symptoms and biochemical deviations. Although unicentric Castleman disease is curable with complete surgical excision, its multicentric counterpart is a considerable therapeutic challenge. The recent development of biological agents, particularly monoclonal antibodies to interleukin-6 and its receptor, allow for more targeted, disease-specific intervention that promises improved response rates and more durable disease control. Imunomodulatory drugs such as thalidomide, lenalidomide and rituximab appear effective in this disease and in some cases even anakinra can induce remission.

Key words:

Castleman disease – POEMS syndrome – TAFRO syndrome – rituximab – thalidomide – lenalidomide – siltuximab – anakinra


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