International evidence-based consensus diagnostic and treatment guidelines for unicentric Castleman disease.
Journal
Blood advances
ISSN: 2473-9537
Titre abrégé: Blood Adv
Pays: United States
ID NLM: 101698425
Informations de publication
Date de publication:
08 12 2020
08 12 2020
Historique:
received:
03
09
2020
accepted:
09
10
2020
entrez:
7
12
2020
pubmed:
8
12
2020
medline:
15
5
2021
Statut:
ppublish
Résumé
Castleman disease (CD) includes a group of rare and heterogeneous disorders with characteristic lymph node histopathological abnormalities. CD can occur in a single lymph node station, which is referred to as unicentric CD (UCD). CD can also involve multicentric lymphadenopathy and inflammatory symptoms (multicentric CD [MCD]). MCD includes human herpesvirus-8 (HHV-8)-associated MCD, POEMS-associated MCD, and HHV-8-/idiopathic MCD (iMCD). The first-ever diagnostic and treatment guidelines were recently developed for iMCD by an international expert consortium convened by the Castleman Disease Collaborative Network (CDCN). The focus of this report is to establish similar guidelines for the management of UCD. To this purpose, an international working group of 42 experts from 10 countries was convened to establish consensus recommendations based on review of treatment in published cases of UCD, the CDCN ACCELERATE registry, and expert opinion. Complete surgical resection is often curative and is therefore the preferred first-line therapy, if possible. The management of unresectable UCD is more challenging. Existing evidence supports that asymptomatic unresectable UCD may be observed. The anti-interleukin-6 monoclonal antibody siltuximab should be considered for unresectable UCD patients with an inflammatory syndrome. Unresectable UCD that is symptomatic as a result of compression of vital neighboring structures may be rendered amenable to resection by medical therapy (eg, rituximab, steroids), radiotherapy, or embolization. Further research is needed in UCD patients with persisting constitutional symptoms despite complete excision and normal laboratory markers. We hope that these guidelines will improve outcomes in UCD and help treating physicians decide the best therapeutic approach for their patients.
Identifiants
pubmed: 33284946
pii: S2473-9529(20)32031-0
doi: 10.1182/bloodadvances.2020003334
pmc: PMC7724917
doi:
Substances chimiques
Antineoplastic Agents
0
Rituximab
4F4X42SYQ6
Banques de données
ClinicalTrials.gov
['NCT02817997', 'NCT03933904']
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
6039-6050Subventions
Organisme : NHLBI NIH HHS
ID : R01 HL141408
Pays : United States
Informations de copyright
© 2020 by The American Society of Hematology.
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