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

The DOI matches its Crossref record; 101 of the 104 references it cites were found in a registry or an index.

Overview of Castleman disease

Open access
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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Research works printed but not linked: 2

  1. Flendrig, 1969, Benign giant lymphoma: the clinical signs and symptoms and the morphological aspects.y, Folia Med Neerl, 12, 119
  2. Takai, 2010, Thrombocytopenia with mild bone marrow fibrosis accompanied by fever, pleural effusion, ascites and hepatosplenomegaly [in Japanese], Rinsho Ketsueki, 51, 320

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Metadata

Authors
Dispenzieri, A., & Fajgenbaum, D. C.
Affiliations
  • Mayo Clinic (US)
  • University of Pennsylvania (US)
ORCID
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
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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