Abstract |
Multicentric Castleman's disease (MCD) is a rare lymphoproliferative disorder presenting with heterogeneous clinical features and with a complex etiology. MCD incidence is increased in people living with HIV/ AIDS when it is causally associated with Kaposi's sarcoma-associated herpes virus (KSHV). HIV-seronegative individuals present with either idiopathic or KSHV-associated MCD. Central to MCD pathology is altered expression and signaling of IL-6, which promotes B-cell proliferation and causes systemic manifestations. KSHV encodes a viral homolog of human IL-6, accounting for its role in MCD, while recent evidence shows an association between IL-6 receptor polymorphisms and idiopathic MCD. The increased understanding of mechanisms underlying the pathogenesis of MCD has guided the use of new monoclonal antibody therapies for treating this complex disorder.
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Authors | Rebecca C Robey, Salvinia Mletzko, Charlotte Colley, Kirsty Balachandran, Mark Bower |
Journal | Immunotherapy
(Immunotherapy)
Vol. 6
Issue 2
Pg. 211-9
( 2014)
ISSN: 1750-7448 [Electronic] England |
PMID | 24491093
(Publication Type: Journal Article, Review)
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Chemical References |
- Antibodies, Monoclonal
- Interleukin-6
- Receptors, Interleukin-6
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Topics |
- Antibodies, Monoclonal
(therapeutic use)
- B-Lymphocytes
(immunology, virology)
- Castleman Disease
(epidemiology, etiology, therapy)
- Cell Proliferation
- Genetic Predisposition to Disease
- HIV Infections
(epidemiology)
- Herpesviridae Infections
(complications, epidemiology)
- Herpesvirus 8, Human
(physiology)
- Humans
- Immunotherapy
- Interleukin-6
(metabolism)
- Polymorphism, Genetic
- Receptors, Interleukin-6
(genetics)
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