Cited by 487Viral-associated cancers and disordersLymphadenopathy Diagnosis and AnalysisIgG4-Related and Inflammatory Diseases

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Overview of Castleman disease

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Dispenzieri, Angela, Fajgenbaum, David C.·2020·Blood

DOI 10.1182/blood.2019000931

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Abstract

Castleman disease (CD) describes a group of at least 4 disorders that share a spectrum of characteristic histopathological features but have a wide range of etiologies, presentations, treatments, and outcomes. CD includes unicentric CD (UCD) and multicentric CD (MCD), the latter of which is divided into idiopathic MCD (iMCD), human herpes virus-8 (HHV8)-associated MCD (HHV8-MCD), and polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS)-associated MCD (POEMS-MCD). iMCD can be further subclassified into iMCD–thrombocytopenia, ascites, reticulin fibrosis, renal dysfunction, organomegaly (iMCD-TAFRO) or iMCD–not otherwise specified (iMCD-NOS). Advances in diagnosis, classification, pathogenesis, and therapy are substantial since the original description of UCD by Benjamin Castleman in 1954. The advent of effective retroviral therapy and use of rituximab in HHV8-MCD have improved outcomes in HHV8-MCD. Anti–interleukin-6–directed therapies are highly effective in many iMCD patients, but additional therapies are required for refractory cases. Much of the recent progress has been coordinated by the Castleman Disease Collaborative Network (CDCN), and further progress will be made by continued engagement of physicians, scientists, and patients. Progress can also be facilitated by encouraging patients to self-enroll in the CDCN’s ACCELERATE natural history registry (#NCT02817997; www.CDCN.org/ACCELERATE).

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Authors: Dispenzieri, A., & Fajgenbaum, D. C.
Affiliations: Mayo Clinic (US)
University of Pennsylvania (US)
ORCID: Dispenzieri, Angela
Fajgenbaum, David C.
Venue: Blood
Publisher: American Society of Hematology
Published: 2020-04-16
Volume: 135
Issue: 16
Pages: 1353-1364
ISSN: 0006-4971 (ISSN-L)
1528-0020
DOI: 10.1182/blood.2019000931
OpenAlex: W3006713791
Cited by: 487
References: 104
Citations by year: 2026: 69 · 2025: 90 · 2024: 90 · 2023: 81 · 2022: 91 · 2021: 52 · 2020: 13
FWCI: 6.36
Type: journal article
Language: en
Open access: green
pubmed.ncbi.nlm.nih.gov
Locations: Blood (publishedVersion)
PubMed (publishedVersion)
PubMed Central (submittedVersion)
Funders: National Institutes of Health (2r01hl141408-06)
National Heart, Lung, and Blood Institute (R01-HL141408)
Topics: Health Sciences › Medicine › Oncology › Viral-associated cancers and disorders
Health Sciences › Medicine › Pathology and Forensic Medicine › Lymphadenopathy Diagnosis and Analysis
Health Sciences › Medicine › Rheumatology › IgG4-Related and Inflammatory Diseases
Keywords: Castleman disease, unicentric Castleman disease, multicentric Castleman disease, idiopathic multicentric Castleman disease, POEMS syndrome, rituximab, disease classification, pathogenesis
MeSH terms: Rituximab, Interleukin-6 Inhibitors, Animals, Castleman Disease, Herpesviridae Infections, Humans, Immunologic Factors, Herpesvirus 8, Human

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