Castleman disease.
Journal
Nature reviews. Disease primers
ISSN: 2056-676X
Titre abrégé: Nat Rev Dis Primers
Pays: England
ID NLM: 101672103
Informations de publication
Date de publication:
25 11 2021
25 11 2021
Historique:
accepted:
06
10
2021
entrez:
26
11
2021
pubmed:
27
11
2021
medline:
18
3
2022
Statut:
epublish
Résumé
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.
Identifiants
pubmed: 34824298
doi: 10.1038/s41572-021-00317-7
pii: 10.1038/s41572-021-00317-7
pmc: PMC9584164
mid: NIHMS1841719
doi:
Substances chimiques
Antibodies, Monoclonal
0
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
84Subventions
Organisme : NCI NIH HHS
ID : R01 CA163217
Pays : United States
Organisme : NCI NIH HHS
ID : R01 CA239583
Pays : United States
Organisme : FIC NIH HHS
ID : R25 TW011215
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL141408
Pays : United States
Organisme : NCI NIH HHS
ID : U54 CA254564
Pays : United States
Organisme : NCI NIH HHS
ID : UM1 CA121947
Pays : United States
Organisme : NCI NIH HHS
ID : R01 CA096500
Pays : United States
Organisme : NIDCR NIH HHS
ID : R01 DE028211
Pays : United States
Organisme : NCI NIH HHS
ID : U01 CA121947
Pays : United States
Informations de copyright
© 2021. Springer Nature Limited.
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