Abstract |
Castleman disease (CD), a heterogeneous group of disorders that share morphological features, is divided into unicentric CD and multicentric CD (MCD) according to the clinical presentation and disease course. Unicentric CD involves a solitary enlarged lymph node and mild symptoms and excision surgery is often curative. MCD includes a form associated with Kaposi sarcoma herpesvirus (KSHV) (also known as human herpesvirus 8) and a KSHV-negative idiopathic form (iMCD). iMCD can present in association with severe syndromes such as TAFRO ( thrombocytopenia, ascites, fever, reticulin fibrosis and organomegaly) or POEMS ( polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder and skin changes). KSHV-MCD often occurs in the setting of HIV infection or another cause of immune deficiency. The interplay between KSHV and HIV elevates the risk for the development of KSHV-induced disorders, including KSHV-MCD, KSHV-lymphoproliferation, KSHV inflammatory cytokine syndrome, primary effusion lymphoma and Kaposi sarcoma. A CD diagnosis requires a multidimensional approach, including clinical presentation and imaging, pathological features, and molecular virology. B cell-directed monoclonal antibody therapy is the standard of care in KSHV-MCD, and anti-IL-6 therapy is the recommended first-line therapy and only treatment of iMCD approved by the US FDA and EMA.
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Authors | Antonino Carbone, Margaret Borok, Blossom Damania, Annunziata Gloghini, Mark N Polizzotto, Raj K Jayanthan, David C Fajgenbaum, Mark Bower |
Journal | Nature reviews. Disease primers
(Nat Rev Dis Primers)
Vol. 7
Issue 1
Pg. 84
(11 25 2021)
ISSN: 2056-676X [Electronic] England |
PMID | 34824298
(Publication Type: Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't, Review)
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Copyright | © 2021. Springer Nature Limited. |
Chemical References |
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Topics |
- Antibodies, Monoclonal
(therapeutic use)
- Castleman Disease
(diagnosis, drug therapy, pathology)
- HIV Infections
(complications, drug therapy, epidemiology)
- Herpesvirus 8, Human
- Humans
- Sarcoma, Kaposi
(diagnosis, epidemiology)
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