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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

1,089

Distinct authors in sample

Gene link

—

Readiness

4/6

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.”

4/6 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 characterisedPresent

    60 HPO annotations (e.g. Myelofibrosis; Elevated vascular endothelial growth factor level; Reduced circulating lactate dehydrogenase concentration) Source

  4. Animal modelNot found

    No Alliance genotype “model of” associations via Monarch for these Mondo IDs

  5. Orphan designationPartial

    1 FDA · 1 EMA designations (none yet with FDA orphan-indication approval) — e.g. chimeric anti-interleukin-6 monoclonal antibody (siltuximab) Source

  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)

60

Associated phenotypes · MONDO:0015564

  • Myelofibrosis
  • Elevated vascular endothelial growth factor level
  • Reduced circulating lactate dehydrogenase concentration
  • Hemophagocytosis
  • Thrombocytopenia

Showing 5 of 60 — open Monarch for the full list.

Animal models (Monarch / Alliance)

None returned for this Mondo ID. Empty here is not proof that no model organism work exists under another name or gene.

Monarch fetch 2026-07-29

Therapies

Designations, candidates, and chemicals

FDA OOPD and EMA orphan designations, Open Targets clinical candidates, and CTD chemical associations via MyDisease.info. These never change the interventional-trial headline.

Orphan designation (FDA · EMA)

2

Designations · no FDA orphan-indication approval yet

  • EMA chimeric anti-interleukin-6 monoclonal antibody (siltuximab)Treatment of Castleman's disease · 30/11/2007 · ExpiredEMA designation
  • FDA siltuximab (SYLVANT)Castleman's Disease · 2006-05-26

Sources: FDA OOPD · EMA orphan designations

Open Targets candidates

11

Drugs / clinical candidates · MONDO_0015564

CTD chemicals (MyDisease.info)

1 associated chemical · 54 pathways. Therapeutic evidence is listed first when present — not a treatment recommendation.

  • Thalidomide · therapeutic

Pathways: EGFR tyrosine kinase inhibitor resistance; Antifolate resistance; Cytokine-cytokine receptor interaction; HIF-1 signaling pathway; FoxO signaling pathway; PI3K-Akt signaling pathway; Toll-like receptor signaling pathway; NOD-like receptor signaling pathway

MyDisease.info · MONDO:0015564

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=3967) is 59.

9,309 papers since the earliest indexed year in this search — median last-10-year count for a rare disease in this dataset is 59 (publications denominator n=3967).

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?

1,089

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 L13 papers · 2026

    Hematology Center, Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Disease in Children, Ministry of Education, Department of Hematology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Nanlishi Road No. 56, Xicheng District, Beijing, 100045, China.

    Papers in Europe PMC
  2. 02
    Wang Y10 papers · 2026

    Department of Hematology, The First Affiliated Hospital of Shandong First Medical University and Shandong Provincial Qianfoshan Hospital, Jinan, China.

    Papers in Europe PMC
  3. 03
    Fajgenbaum DC7 papers · 2026

    Department of Medicine, Center for Cytokine Storm Treatment & Laboratory, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.

    Papers in Europe PMC
  4. 04
    Koga T7 papers · 2026

    Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, JPN.

    Papers in Europe PMC
  5. 05
    Li J7 papers · 2026

    Department of Pathology, Xi'an Children's Hospital, Xi'an Jiaotong University, Xi'an 710043, China.

    Papers in Europe PMC
  6. 06
    Nishimura Y7 papers · 2026

    Mayo Clinic, Rochester, Minnesota, USA.

    Papers in Europe PMC
  7. 07
    Kawakami A6 papers · 2026

    Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, JPN.

    Papers in Europe PMC
  8. 08
    Liu J6 papers · 2026

    Department of Rheumatology and Immunology, The Affiliated Hospital of Guizhou Medical University, Guiyang, China.

    Papers in Europe PMC
  9. 09
    Sumiyoshi R6 papers · 2026

    Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, JPN.

    Papers in Europe PMC
  10. 10
    van Rhee F6 papers · 2026

    University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA.

    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 11 September 2026

26 interventional trials — more than 77.2% of diseases in the trials denominator have none at all (5501 of 7126; this disease is at the 95.6th percentile).

low confidence · 95.6th 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

Other registries (secondary)

Broader net from EU CTIS, ISRCTN, and ICTRP when available — deduped against ClinicalTrials.gov IDs already counted above. Dual-model LLM relevance gates what we keep. These rows are not added to the interventional headline.

raw 3 · after dedupe 3 · already on CT.gov 0 · kept 0 · parent 0 · uncertain 3 · dropped 0 · fetched 2026-07-29

Source notes: ictrp: Error: ICTRP public search unavailable (WHO portal is SPA-only; SOAP needs partnership). Tried: https://apps.who.int/tri

No secondary-registry studies passed dual-model relevance for this condition name (after dedupe).

Uncertain / not reviewed (3)

Where to find support

Condition-specific patient organisations, when Orphanet lists them, are on the disease’s Orphanet page. We also link umbrella groups that support undiagnosed and ultra-rare families.

Orphanet entry for Castleman disease — check Associations / patient organisations on that page.

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