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ORPHA:160

Castleman disease

low confidenceDisorder

Also known as: Angiofollicular ganglionic hyperplasia · Angiofollicular lymph hyperplasia

Publications

9,309

Trials

26

Interventional, condition-specific

Researchers

1,157

Distinct authors in sample

Gene link

—

Readiness

2/6

Stages with a signal

Clinical definition (Orphanet)

A rare lymphoid hemopathy characterized by involvement of lymph nodes in any part of the body, most frequently the mediastinum, abdomen, neck, or spleen, and occurring as unicentric, multicentric, or KSHV/HHV8-associated multicentric Castleman disease. Depending on the type, patients are most commonly asymptomatic or typically present with systemic symptoms.

How rare: How common this is has not been clearly measured.

Orphanet entry

Cross-references

Joined from Mondo / Orphanet. MeSH labels may enter searches; UMLS / OMIM / NCIT are stored for reference.

Additional Mondo synonyms (10)

AFLH · Castleman's disease · Castleman's tumor · Castleman's tumour · GLNH · angiofollicular ganglionic hyperplasia · angiofollicular lymph hyperplasia · angiofollicular lymph node hyperplasia · angiofollicular lymphoid hyperplasia · giant lymph node hyperplasia

Research stages

Trial readiness signals

Where this condition sits on an open-data research pipeline — not how close a treatment is, and not medical advice. Empty stages often mean “not in these databases under this Mondo ID,” not “impossible.”

2/6 stages with a signal

An interventional trial matched this condition name on ClinicalTrials.gov — see trials below.

  1. Gene identifiedNot found

    No GenCC disease–gene assertion in this build

  2. LiteraturePresent

    9,309 matched papers (4,366 in last 10 years) Source

  3. Phenotype characterisedNot checked

    Not yet enriched from Monarch / HPO

  4. Animal modelNot checked

    Not yet enriched from Monarch / Alliance

  5. Orphan designationNot checked

    FDA/EMA orphan-drug designation not enriched yet

  6. Interventional trialPresent

    26 matched on ClinicalTrials.gov (4 recruiting in sample)

Biology

Genes and phenotypes

Gene–disease validity from GenCC, plus phenotypes and animal models joined from Monarch Initiative via Mondo ID — not a clinical diagnosis aid.

Do we know what causes it?

Not yet — the cause hasn't been pinned down in GenCC.

No strong gene–disease assertion joined for this Orphanet entity.

Phenotypes (Monarch / HPO)

Not enriched in this build — Monarch phenotype joins were not run for this record.

Animal models (Monarch / Alliance)

Not enriched in this build.

Literature

Is anyone studying this?

9,309

9,309 papers — among the better-studied rare conditions, though still a fraction of common-disease literature (breast cancer: over 700,000). Median papers in the last 10 years for a rare disease in this dataset (publications denominator n=5449) is 41.

9,309 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 41 (publications denominator n=5449).

4,366 in the last 10 years · low confidence

Phrase hits: 9,309 · MeSH hits: 0

Open Europe PMC search

Who's working on it?

1,157

Distinct author names in 200 sampled papers — named people below.

Who's working on it?

People publishing on this condition (sampled Europe PMC records). Affiliation is the most recent found in that sample.

  1. 01
    Zhang L8 papers · 2026

    Department of Hematology, Peking Union Medical College Hospital,Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.

    Papers in Europe PMC
  2. 02
    Li J6 papers · 2026

    Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China. lijian@pumch.cn.

    Papers in Europe PMC
  3. 03
    Liu J6 papers · 2026

    Department of Urology, Hebei General Hospital, Shijiazhuang, Hebei, China.

    Papers in Europe PMC
  4. 04
    Wang Y6 papers · 2026

    Department of Oral and Maxillofacial Surgery, Yantai Affiliated Hospital of Binzhou Medical University, Yantai, China.

    Papers in Europe PMC
  5. 05
    Li Y5 papers · 2026

    Department of Pharmacy, Nanfang Hospital, Southern Medical University, Guangzhou, China.

    Papers in Europe PMC
  6. 06
    Liu W5 papers · 2026

    Department of Nephrology, Peking University Shenzhen Hospital, Shenzhen, Guangdong Province, 518000, China.

    Papers in Europe PMC
  7. 07
    Dispenzieri A4 papers · 2026

    Mayo Clinic, Rochester, Minnesota, USA.

    Papers in Europe PMC
  8. 08
    Li X4 papers · 2025

    Department of Hematology, Beijing Tongren Hospital, Capital Medical University, Beijing, China.

    Papers in Europe PMC
  9. 09
    Li Z4 papers · 2025

    Department of Stomatology, Jinling Hospital, Affiliated Hospital of Medical School, Nanjing University, 305 East Zhongshan Road, Nanjing, 210012, Jiangsu, China.

    Papers in Europe PMC
  10. 10
    Wang S4 papers · 2026

    Department of Dermatology and Venereology, Peking University First Hospital, Beijing, China.

    Papers in Europe PMC

Clinical research

Is a treatment being tested?

26

interventional trials for this specific condition

26 interventional trials matched this specific condition name; 4 currently recruiting in our sample.

Data as of 27 July 2026

26 interventional trials — more than 73% of diseases in the trials denominator have none at all (5114 of 7003; this disease is at the 95.3th percentile).

low confidence · 95.3th percentile (trials denominator)

Recruiting interventional trials

From the matched ClinicalTrials.gov set

26 interventional trials matched after quoted-phrase search and title/condition post-filter.

Observational and natural-history studies

15 observational studies match this condition. These do not test a treatment and are not counted in the interventional-trial headline, but they are genuine research: natural-history work often defines the endpoints needed for a future rare-disease trial, and families may be able to enroll.

Recruiting or not-yet-recruiting

Open the complete matched search on ClinicalTrials.gov

Where to find support

We do not yet link condition-specific patient organisations. These umbrella groups support undiagnosed and ultra-rare families:

India — NPRD

Last verified 2026-07-26

This ORPHAcode is not on our curated NPRD list (direct or Mondo-parent match). That does not decide clinical eligibility; families in India should ask a notified Centre of Excellence about current coverage.

Hand-curated for this project. ORPHAcode mappings are best-effort and may be incomplete or imprecise for umbrella categories. Parent (Mondo) matches mean the policy lists a broader category — confirm eligibility with a Centre of Excellence. Financial entitlements summarised from public policy statements and may change. This is not official government guidance.

How we counted this

Europe PMC query (preferred label + any corrected label + Orphanet and Mondo exact synonyms, stoplisted; unioned with resolved MeSH labels when available). UMLS / OMIM / NCIT cross-references are stored on the overview but are not added to the query string.

"Castleman disease" OR "Angiofollicular ganglionic hyperplasia" OR "Angiofollicular lymph hyperplasia" OR "Castleman's disease" OR "Castleman's tumor" OR "Castleman's tumour" OR "angiofollicular lymph node hyperplasia" OR "angiofollicular lymphoid hyperplasia" OR "giant lymph node hyperplasia"

Run this search on Europe PMC

ClinicalTrials.gov query (quoted phrases + MeSH via query.cond, plus recall-expansion terms when used):

"Castleman disease" OR "Angiofollicular ganglionic hyperplasia" OR "Angiofollicular lymph hyperplasia" OR "Castleman's disease" OR "Castleman's tumor" OR "Castleman's tumour" OR "angiofollicular lymph node hyperplasia" OR "angiofollicular lymphoid hyperplasia" OR "giant lymph node hyperplasia"

Interventional trials matched via: phrase (mesh = registered under a MeSH descriptor no name phrase would catch; recall-expansion = gene / selected parent terms used only for trials).

Study-type breakdown: 26 interventional · 15 observational · 0 expanded access. Only interventional studies enter the trial headline.

Query health: ok — strategies attempted: phrase; with hits: phrase

Run this search on ClinicalTrials.gov

Synonyms dropped by stoplist: AFLH; GLNH

Confidence reasoning

  • Preferred label is short or not clearly distinctive
  • 2 synonym(s) dropped by stoplist (may under-count)
  • No label/synonym collisions with other diseases in this corpus

Ingested 2026-07-26T12:42:20.629Z