open access

Vol 52, No 1 (2021)
Review article
Submitted: 2021-02-07
Accepted: 2021-02-07
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Idiopathic multicentric Castleman disease: pathogenesis, clinical presentation and recommendations for treatment based on the Castleman Disease Collaborative Network (CDCN)

Beata Ostrowska, Joanna Romejko-Jarosińska, Katarzyna Domańska-Czyż, Jan Walewski
DOI: 10.5603/AHP.2021.0005
·
Acta Haematol Pol 2021;52(1):29-37.

open access

Vol 52, No 1 (2021)
REVIEW ARTICLE
Submitted: 2021-02-07
Accepted: 2021-02-07

Abstract

Castleman disease is a very rare, lymphoproliferative disease, driven by dysregulation of the cytokine interleukin 6 (IL-6) and other proinflammatory cytokines with the development of symptoms of systemic inflammation, reactive proliferation of lymphocytes, and damage to numerous organs. HHV-8 infection plays a major role in the pathogenesis of human immunodeficiency virus (HIV+) multicentric Castleman disease (MCD). The aetiology of the idiopathic form of MCD (HIV/HHV-8 negative) is unknown. In 2017, a panel of Castleman Disease Collaborative Network (CDCN) experts developed unified diagnostic criteria necessary for the diagnosis of idiopathic MCD. The disease has a diverse course, with a broad spectrum from mild to severe. The drug of choice according to the CDCN recommendation of 2018 for patients requiring treatment is anti-IL-6 monoclonal antibody, siltuximab. Immunomodulatory and immunosuppressive drugs are an alternative first-line treatment when anti-IL-6/IL-6R drugs are unavailable, and in subsequent lines of treatment after the failure of anti-IL-6 antibodies. Chemotherapy is reserved for the treatment of severe forms of the disease. The prognosis in most patients is moderately good. Unfortunately, the availability of the new therapies remains limited.

Abstract

Castleman disease is a very rare, lymphoproliferative disease, driven by dysregulation of the cytokine interleukin 6 (IL-6) and other proinflammatory cytokines with the development of symptoms of systemic inflammation, reactive proliferation of lymphocytes, and damage to numerous organs. HHV-8 infection plays a major role in the pathogenesis of human immunodeficiency virus (HIV+) multicentric Castleman disease (MCD). The aetiology of the idiopathic form of MCD (HIV/HHV-8 negative) is unknown. In 2017, a panel of Castleman Disease Collaborative Network (CDCN) experts developed unified diagnostic criteria necessary for the diagnosis of idiopathic MCD. The disease has a diverse course, with a broad spectrum from mild to severe. The drug of choice according to the CDCN recommendation of 2018 for patients requiring treatment is anti-IL-6 monoclonal antibody, siltuximab. Immunomodulatory and immunosuppressive drugs are an alternative first-line treatment when anti-IL-6/IL-6R drugs are unavailable, and in subsequent lines of treatment after the failure of anti-IL-6 antibodies. Chemotherapy is reserved for the treatment of severe forms of the disease. The prognosis in most patients is moderately good. Unfortunately, the availability of the new therapies remains limited.

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Keywords

multicentric Castleman disease, interleukin 6, siltuximab

About this article
Title

Idiopathic multicentric Castleman disease: pathogenesis, clinical presentation and recommendations for treatment based on the Castleman Disease Collaborative Network (CDCN)

Journal

Acta Haematologica Polonica

Issue

Vol 52, No 1 (2021)

Article type

Review article

Pages

29-37

DOI

10.5603/AHP.2021.0005

Bibliographic record

Acta Haematol Pol 2021;52(1):29-37.

Keywords

multicentric Castleman disease
interleukin 6
siltuximab

Authors

Beata Ostrowska
Joanna Romejko-Jarosińska
Katarzyna Domańska-Czyż
Jan Walewski

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