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Overview of Castleman disease
Open accessAbstract
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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“Patients with HHV-8–associated multicentric Castleman’s disease are immunocompromised as a result of human immunodeficiency virus infection or a genetic susceptibility, making it difficult to control the HHV-8 infection, which is a common, typically asymptomatic infection in the general population.69”
From Cytokine storm (2020) · cites this paper as [69]
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Metadata
- Authors
- Dispenzieri, A., & Fajgenbaum, D. C.
- Affiliations
- Mayo Clinic (US)
- University of Pennsylvania (US)
- 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
- 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
- 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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