Definition and research guide
This page combines a MeSH definition, publication statistics and selected research abstracts. It is a starting point for reading the literature, not a medical textbook or a systematic evidence review.
Content available: 4 complete abstracts (799 words), 10 references. Full article text is not stored or reproduced here; follow the PMID, DOI or PMC links to read the original sources and their access terms.
Definition
Large benign, hyperplastic lymph nodes. The more common hyaline vascular subtype is characterized by small hyaline vascular follicles and interfollicular capillary proliferations. Plasma cells are often present and represent another subtype with the plasma cells containing IgM and immunoglobulin A.
Definition: NLM Medical Subject Headings (MeSH) scope note (Public domain (U.S. National Library of Medicine); attribution requested). View the MeSH record.
Also known as: Giant Lymph Node Hyperplasia; Angiofollicular Lymphoid Hyperplasia; Angiofollicular Lymphoid Hyperplasias; Hyperplasia, Angiofollicular Lymphoid; Lymphoid Hyperplasia, Angiofollicular; Castleman's Disease; Castleman's Tumor; Castlemans Tumor.
Where it fits in the MeSH classification
- Broader topics
- Lymphoproliferative Disorders
Related concepts
- Immunoglobulin
Research landscape
PubMed indexes 3,324 records tagged with the MeSH heading “Castleman Disease” (counts retrieved 2026-10-29). 768 of them (23.1%) were published from 2020 onward, and the busiest period so far is 2010-2019 with 1,007 records.
Reviews account for 605 records (18.2%) and meta-analyses for 4. Randomized controlled trials number 15 (0.5%) as a share of all records on the topic. Randomized trials make up only a small share of this literature, so much of what is known comes from laboratory, observational or descriptive work.
Show the data
| Period | Records |
|---|---|
| through 1999 | 705 |
| 2000-2009 | 844 |
| 2010-2019 | 1,007 |
| 2020 onward | 768 |
Publication-type chart data
| Type | Records | Share |
|---|---|---|
| Reviews | 605 | 18.2% |
| Meta-analyses | 4 | 0.1% |
| Randomized controlled trials | 15 | 0.5% |
Method: PubMed query "Castleman Disease"[MeSH Terms] with publication-type and date filters, retrieved 2026-10-29.
Reading the evidence in immunology
Immune responses are studied in model organisms, in cells from donors and in patients, and the same pathway can behave differently across these settings. Mouse immune systems differ from human ones in important ways, and blood measurements may not reflect what happens in tissues. Clinical conclusions about immune-mediated disease or immunotherapy come mainly from controlled trials and long-term cohorts. Check the species and tissue studied, whether markers were linked to outcomes patients notice, and the follow-up time.
Evidence from open-access studies
These open-access studies come from PubMed’s best-match results for this heading (papers whose main topic it is), ranked here by citation count, with one recent addition. Each abstract is reproduced in full under its recorded open licence so that you can read the authors’ own words. They illustrate the literature; they are not a systematic summary of it. Citation counts measure attention, not reliability or clinical benefit.
| Study / authors | Year | Source label / design | Europe PMC citations | Licence / source |
|---|---|---|---|---|
| Elevated serum interferon γ-induced protein 10 kDa is associated with TAFRO syndrome | 2017 | Research article Design not recorded in structured metadata | 60 | cc by PMID 28205564 · DOI · PMC5304226 |
| Idiopathic Plasmacytic Lymphadenopathy Forms an Independent Subtype of Idiopathic Multicentric Castleman Disease | 2022 | Research article Design not recorded in structured metadata | 46 | cc by PMID 36142213 · DOI · PMC9499480 |
| Idiopathic multicentric Castleman disease and associated autoimmune and autoinflammatory conditions: practical guidance for diagnosis | 2023 | Review article Design not recorded in structured metadata | 40 | cc by PMID 35997567 · DOI · PMC10070070 |
| Snapshot Look at Castleman Disease | 2026 | Review article Design not recorded in structured metadata | 1 | cc by PMID 41664979 · DOI · PMC12887673 |
Elevated serum interferon γ-induced protein 10 kDa is associated with TAFRO syndrome [1]
Abstract reading summary — source excerpts
- Opening excerpt
- Multicentric Castleman disease (MCD) is a heterogeneous lymphoproliferative disorder.
- Closing excerpt
- There was a strong correlation between high serum IP-10 and the presence of TAFRO-iMCD, suggesting that IP-10 might be involved in the pathogenesis of TAFRO-iMCD.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (197 words)
Multicentric Castleman disease (MCD) is a heterogeneous lymphoproliferative disorder. It is characterized by inflammatory symptoms, and interleukin (IL)-6 contributes to the disease pathogenesis. Human herpesvirus 8 (HHV-8) often drives hypercytokinemia in MCD, although the etiology of HHV-8-negative MCD is idiopathic (iMCD). A distinct subtype of iMCD that shares a constellation of clinical features including thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O) has been reported as TAFRO-iMCD, however the differences in cytokine profiles between TAFRO-iMCD and iMCD have not been established. We retrospectively compared levels of serum interferon γ-induced protein 10 kDa (IP-10), platelet-derived growth factor (PDGF)-AA, interleukin (IL)-10, and other cytokines between 11 cases of TAFRO-iMCD, 6 cases of plasma cell type iMCD, and 21 healthy controls. During flare-ups, patients with TAFRO-iMCD had significantly higher serum IP-10 and tended to have lower PDGF-AA levels than the other 2 groups. In addition, serum IL-10, IL-23, and vascular endothelial growth factor-A were elevated in both TAFRO-iMCD and iMCD. Elevated serum IP-10 is associated with inflammatory diseases including infectious diseases. There was a strong correlation between high serum IP-10 and the presence of TAFRO-iMCD, suggesting that IP-10 might be involved in the pathogenesis of TAFRO-iMCD.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 28205564 · DOI · PMC5304226
Idiopathic Plasmacytic Lymphadenopathy Forms an Independent Subtype of Idiopathic Multicentric Castleman Disease [2]
Abstract reading summary — source excerpts
- Opening excerpt
- Idiopathic multicentric Castleman disease (iMCD) is a type of Castleman disease that is not related to KSHV/HHV8 infection.
- Closing excerpt
- Thus, the IPL group is a clinicopathologically uniform entity that forms an independent subtype of iMCD.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (245 words)
Idiopathic multicentric Castleman disease (iMCD) is a type of Castleman disease that is not related to KSHV/HHV8 infection. Currently, iMCD is classified into iMCD-TAFRO (thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly) and iMCD-NOS (not otherwise specified). The former has been established as a relatively homogeneous disease unit that has been recently re-defined, while the latter is considered to be a heterogeneous disease that could be further divided into several subtypes. In 1980, Mori et al. proposed the concept of idiopathic plasmacytic lymphadenopathy (IPL), a disease presenting with polyclonal hypergammaglobulinemia and a sheet-like proliferation of mature plasma cells in the lymph nodes. Some researchers consider IPL to be a part of iMCD-NOS, although it has not been clearly defined to date. This is the first paper to analyze iMCD-NOS clinicopathologically, to examine whether IPL forms a uniform disease unit in iMCD. Histologically, the IPL group showed prominent plasmacytosis and the hyperplasia of germinal centers, while the non-IPL group showed prominent vascularity. Clinically, the IPL group showed significant thrombocytosis and elevated serum IgG levels compared to the non-IPL group (p = 0.007, p < 0.001, respectively). Pleural effusion and ascites were less common in the IPL group (p < 0.001). The IPL group was more likely to have an indolent clinical course and a good response to the anti-IL-6 receptor antibody, while the non-IPL counterpart frequently required more aggressive medical interventions. Thus, the IPL group is a clinicopathologically uniform entity that forms an independent subtype of iMCD.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 36142213 · DOI · PMC9499480
Idiopathic multicentric Castleman disease and associated autoimmune and autoinflammatory conditions: practical guidance for diagnosis [3]
Abstract reading summary — source excerpts
- Opening excerpt
- Idiopathic multicentric Castleman disease (iMCD) is an infrequent and life-threatening disorder characterized by systemic inflammatory symptoms, generalized lymphadenopathy, polyclonal lymphocyte proliferation and organ dysfunction caused by a hyperinflammatory state.
- Closing excerpt
- In this review, we explore the overlap between iMCD and inflammatory diseases and provide practical guidance on iMCD diagnosis in order to avoid misdiagnosis and confusion with other autoimmune or autoinflammatory conditions.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (184 words)
Idiopathic multicentric Castleman disease (iMCD) is an infrequent and life-threatening disorder characterized by systemic inflammatory symptoms, generalized lymphadenopathy, polyclonal lymphocyte proliferation and organ dysfunction caused by a hyperinflammatory state. It accounts for one-third to one-half of all multicentric Castleman disease (MCD) cases. iMCD is often associated with autoimmune manifestations that may precede the iMCD diagnosis, be identified at the same time or follow it. In addition, iMCD may also coincide with a number of autoimmune diseases (such as psoriasis or myasthenia gravis) or autoinflammatory diseases (such as familial Mediterranean fever). Moreover, diverse inflammatory disorders, such as rheumatoid arthritis, systemic lupus erythematosus, adult-onset Still disease, systemic juvenile idiopathic arthritis, immunoglobulin (IgG4) related disease, or the recently described VEXAS syndrome, can present clinical features or lymphadenopathy with histopathological 'Castleman-like' findings compatible with those of iMCD. Given the iMCD clinical heterogeneity and the overlap with other autoimmune or autoinflammatory disorders, iMCD diagnosis can be challenging. In this review, we explore the overlap between iMCD and inflammatory diseases and provide practical guidance on iMCD diagnosis in order to avoid misdiagnosis and confusion with other autoimmune or autoinflammatory conditions.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 35997567 · DOI · PMC10070070
Snapshot Look at Castleman Disease [4]
Abstract reading summary — source excerpts
- Opening excerpt
- Castleman disease (CD) is a rare and heterogeneous group of lymphoproliferative disorders characterised by abnormal proliferation of lymphoid tissue.
- Closing excerpt
- Continued research into its molecular underpinnings and targeted treatments is critical to improving patient outcomes and establishing evidence- based guidelines.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (173 words)
Castleman disease (CD) is a rare and heterogeneous group of lymphoproliferative disorders characterised by abnormal proliferation of lymphoid tissue. First described in the 1950s, it has since been classified into two major clinical forms: unicentric CD (UCD), involving a single lymph node region and multicentric CD (MCD), which affects multiple regions and is often systemic. Further subclassification of MCD includes HHV8-associated MCD, POEMS-associated MCD and idiopathic MCD (iMCD), each with distinct pathophysiologic mechanisms and clinical implications. This review summarises current understanding of the epidemiology, clinical presentation, histopathology, pathogenesis and diagnostic challenges of CD. It also explores recent advances in molecular biology, including the role of interleukin-6 (IL-6), human herpesvirus-8 (HHV8) and aberrant immune signalling in disease progression. Therapeutic strategies vary significantly depending on the subtype and range from surgical resection in UCD to immunotherapy, siltuximab and cytotoxic chemotherapy in MCD. Despite progress, CD remains underdiagnosed and poorly understood, especially in its idiopathic forms. Continued research into its molecular underpinnings and targeted treatments is critical to improving patient outcomes and establishing evidence- based guidelines.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 41664979 · DOI · PMC12887673
Key reviews and meta-analyses
The most cited review-type papers among PubMed’s best-match results for this heading, listed with bibliographic details only. Follow the links for the full text and access terms.
- Research article International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease [5]
- Research article Overview of Castleman disease [6]
- Research article Interleukin-6 as a therapeutic target [7]
- Research article The management of unicentric and multicentric Castleman's disease: a report of 16 cases and a review of the literature [8]
- Research article Surgery in Castleman's disease: a systematic review of 404 published cases [9]
- Research article HHV-8-negative, idiopathic multicentric Castleman disease: novel insights into biology, pathogenesis, and therapy [10]
References
- Iwaki N, Gion Y, Kondo E, Kawano M, Masunari T, Moro H, et al.. Elevated serum interferon γ-induced protein 10 kDa is associated with TAFRO syndrome. . 2017. PMID 28205564 · DOI · PMC5304226
- Nishikori A, Nishimura MF, Nishimura Y, Otsuka F, Maehama K, Ohsawa K, et al.. Idiopathic Plasmacytic Lymphadenopathy Forms an Independent Subtype of Idiopathic Multicentric Castleman Disease. . 2022. PMID 36142213 · DOI · PMC9499480
- González García A, Fernández-Martín J, Robles Marhuenda Á. Idiopathic multicentric Castleman disease and associated autoimmune and autoinflammatory conditions: practical guidance for diagnosis. . 2023. PMID 35997567 · DOI · PMC10070070
- Jitaru C, Zlampa N, Dima D, Bojan A, Zdrenghea M, Urian L, et al.. Snapshot Look at Castleman Disease. . 2026. PMID 41664979 · DOI · PMC12887673
- Fajgenbaum DC, Uldrick TS, Bagg A, Frank D, Wu D, Srkalovic G, et al.. International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. Blood. 2017. PMID 28087540 · DOI · PMC5364342
- Dispenzieri A, Fajgenbaum DC. Overview of Castleman disease. Blood. 2020. PMID 32106302 · DOI · PMC13174927
- Rossi JF, Lu ZY, Jourdan M, Klein B. Interleukin-6 as a therapeutic target. Clin Cancer Res. 2015. PMID 25589616 · DOI
- Bowne WB, Lewis JJ, Filippa DA, Niesvizky R, Brooks AD, Burt ME, et al.. The management of unicentric and multicentric Castleman's disease: a report of 16 cases and a review of the literature. Cancer. 1999. PMID 10091744 · DOI
- Talat N, Belgaumkar AP, Schulte KM. Surgery in Castleman's disease: a systematic review of 404 published cases. Ann Surg. 2012. PMID 22367441 · DOI
- Fajgenbaum DC, van Rhee F, Nabel CS. HHV-8-negative, idiopathic multicentric Castleman disease: novel insights into biology, pathogenesis, and therapy. Blood. 2014. PMID 24622327 · DOI
About this article
- Sources
- NLM Medical Subject Headings (definition, synonyms); NCBI PubMed (counts, identifiers); Europe PMC (open-access abstracts and citation counts).
- Verification
- Every reference was resolved against PubMed on 2026-10-31; title, year and DOI matched.
- Review state
- automated source verified; no human expert review has been recorded yet.
- Version
- 1, last updated 2026-10-29.
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Evidence strength
EvidenceHelix's computed view of the study designs behind this topic: how common meta-analyses, randomized trials and reviews are, how recent the literature is, and a plain-language reading. It describes the design mix of the literature. It is not a quality appraisal of any study, and it is not medical advice.