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Atlas / Disease / Castleman disease

Castleman disease

disease
On this page

Also known as AFLHALNHangiofollicular ganglionic hyperplasiaangiofollicular lymph hyperplasiaangiofollicular lymph node hyperplasiaangiofollicular lymphoid hyperplasiaCastleman's diseaseCastleman's tumorCastleman's tumourgiant lymph node hyperplasiaGLNH

Summary

Castleman disease (MONDO:0015564) is a disease and 22 clinical trials. Top therapeutic interventions include valganciclovir, nelfinavir, and nintedanib. A subtype of lymphoproliferative syndrome — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

  • Prevalence: 1-9 / 100 000 (United States) [Orphanet-validated]
  • Phenotypes (HPO): 29
  • Clinical trials: 22

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

TypeClassValueGeographyValidation
Annual incidence1-9 / 100 000United StatesValidated

Signs & symptoms

Clinical features (HPO)

29 HPO clinical features (Orphanet curated; top 29 by frequency):

HPO IDTermFrequency
HP:0002716LymphadenopathyVery frequent (80-99%)
HP:0001824Weight lossFrequent (30-79%)
HP:0001903AnemiaFrequent (30-79%)
HP:0002027Abdominal painFrequent (30-79%)
HP:0002729Follicular hyperplasiaFrequent (30-79%)
HP:0003565Elevated erythrocyte sedimentation rateFrequent (30-79%)
HP:0011227Elevated circulating C-reactive protein concentrationFrequent (30-79%)
HP:0012378FatigueFrequent (30-79%)
HP:0025142Constitutional symptomFrequent (30-79%)
HP:0030783Increased circulating interleukin 6 concentrationFrequent (30-79%)
HP:0100721Mediastinal lymphadenopathyFrequent (30-79%)
HP:0000952JaundiceOccasional (5-29%)
HP:0002017Nausea and vomitingOccasional (5-29%)
HP:0003270Abdominal distentionOccasional (5-29%)
HP:0008940Generalized lymphadenopathyOccasional (5-29%)
HP:0011024Abnormality of the gastrointestinal tractOccasional (5-29%)
HP:0012735CoughOccasional (5-29%)
HP:0025066Decreased mean corpuscular volumeOccasional (5-29%)
HP:0030157Flank painOccasional (5-29%)
HP:0031500Abdominal massOccasional (5-29%)
HP:0000083Renal insufficiencyVery rare (<1-4%)
HP:0000790HematuriaVery rare (<1-4%)
HP:0001723Restrictive cardiomyopathyVery rare (<1-4%)
HP:0001873ThrombocytopeniaVery rare (<1-4%)
HP:0002094DyspneaVery rare (<1-4%)
HP:0005214Intestinal obstructionVery rare (<1-4%)
HP:0006000Ureteral obstructionVery rare (<1-4%)
HP:0011974MyelofibrosisVery rare (<1-4%)
HP:0012050AnasarcaVery rare (<1-4%)

Identifiers

Disease identifiers

FieldValue
Canonical nameCastleman disease
Mondo IDMONDO:0015564
MeSHD005871
Orphanet160
DOIDDOID:0111157
ICD-10-CMD47.Z2
ICD-111940989685
NCITC3056
SNOMED CT207036003
UMLSC0017531
MedGen42211
GARD0012656
MedDRA10050251
NORD898
Is cancer (heuristic)no

Also known as: AFLH · ALNH · angiofollicular ganglionic hyperplasia · angiofollicular lymph hyperplasia · angiofollicular lymph node hyperplasia · angiofollicular lymphoid hyperplasia · Castleman disease · Castleman’s disease · Castleman’s tumor · Castleman’s tumour · giant lymph node hyperplasia · GLNH

Disease family

This is a subtype of lymphoproliferative syndrome. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › immune system disorder › inborn error of immunity › lymphoproliferative syndrome › Castleman disease

Related subtypes (7): X-linked lymphoproliferative syndrome, Dianzani autoimmune lymphoproliferative disease, lymphoproliferative syndrome 1, lymphoproliferative syndrome 2, autoimmune lymphoproliferative syndrome, severe combined immunodeficiency due to CD70 deficiency, atypical lymphoproliferative disorder

Subtypes (4): pediatric Castleman disease, localized Castleman disease, multicentric Castleman disease, idiopathic multicentric Castleman disease

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

Drugs indicated or in trials for this disease

1 approved drug — disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.

DrugStatus
SiltuximabApproved (phase 4)

11 drugs in clinical trials for this disease (phase 2–3, investigational): efficacy not established — a trial record, not an indication.

DrugHighest phase
BortezomibPhase 2
DexamethasonePhase 2
EtoposidePhase 2
PrednisonePhase 2
RituximabPhase 2
SirolimusPhase 2
ThalidomidePhase 2
TocilizumabPhase 2
ValganciclovirPhase 2
ZanubrutinibPhase 2
ZidovudinePhase 2

Clinical trials & evidence

Clinical trials

Clinical trials: 22.

Phase distribution (across all retrieved trials)

PhaseTrials
Not specified11
PHASE25
PHASE14
PHASE41
EARLY_PHASE11

Top trials by phase / activity

NCTPhaseStatusTitle
NCT00361933PHASE4WITHDRAWNValganciclovir to Treat HHV-8 Associated Multicentric Castleman’s Disease
NCT03933904PHASE2ACTIVE_NOT_RECRUITINGSirolimus in Previously Treated Idiopathic Multicentric Castleman Disease
NCT06643091PHASE2NOT_YET_RECRUITINGNintedanib Treatment in Unicentric Castleman Disease
NCT07085039PHASE2RECRUITINGRuxolitinib in Previously Treated Idiopathic Multicentric Castleman Disease
NCT00127569PHASE2TERMINATEDRituximab in the Treatment of HIV Associated Multicentric Castleman Disease Dependent on Chemotherapy
NCT01441063PHASE2COMPLETEDTocilizumab for KSHV-Associated Multicentric Castleman Disease
NCT00412321PHASE1COMPLETEDA Safety and Efficacy Study of CNTO 328 in Patients With B-Cell Non-Hodgkin’s Lymphoma, Multiple Myeloma, or Castleman’s Disease
NCT00891280PHASE1UNKNOWNDose-escalation Study of Oral CX-4945
NCT01183598PHASE1COMPLETEDA Study to Provide RoActemra/Actemra (Tocilzumab) to Patients With Multicentric Castleman’s Disease Who Demonstrated Benefit From Previous RoActemra/Actemra Treatment
NCT01552434PHASE1TERMINATEDBevacizumab and Temsirolimus Alone or in Combination With Valproic Acid or Cetuximab in Treating Patients With Advanced or Metastatic Malignancy or Other Benign Disease
NCT02080416EARLY_PHASE1TERMINATEDNelfinavir for the Treatment of Gammaherpesvirus-Related Tumors
NCT02817997Not specifiedRECRUITINGInternational Registry for Patients With Castleman Disease
NCT03300830Not specifiedRECRUITINGMolecular Characterization of Viral-associated Tumors, Tumors Occurring in the Setting of HIV or Other Immune Disorders and Castleman Disease
NCT03717844Not specifiedRECRUITINGRegistry for Adults With Plasma Cell Disorders (PCD’s)
NCT05081141Not specifiedRECRUITINGHHV8 and Solid Organ Transplantation
NCT05200715Not specifiedRECRUITINGAutoInflammatory Disease Alliance Registry (AIDA)
NCT05283993Not specifiedRECRUITINGA Cohort Study of Plasma Cell Disorders (PCDs) in PKUFH
NCT06720870Not specifiedRECRUITINGAn Italian Multicenter Retrospective Observational Study to Assess Effectiveness and Safety of Siltuximab for Patients with Castleman’s Disease Treated in Italy in a Real-life Context
NCT02853968Not specifiedCOMPLETEDUnlock the Cell: Castleman’s Disease Flow Cytometry Study
NCT02871050Not specifiedWITHDRAWNCastleman Disease Collaborative Network Biobank
NCT05028621Not specifiedSUSPENDEDMolecular Basis of Langerhans and Non-Langerhans Cell Histiocytic Neoplasms and Castleman Disease
NCT05440305Not specifiedCOMPLETEDUnicentric Form of Castleman Disease - Surgery Therapy Benefit

Drugs tested across these trials (top 30)

MoleculeMax phaseTrials referencing
VALGANCICLOVIR42
NELFINAVIR41
NINTEDANIB41
RITUXIMAB41
SILMITASERTIB21

About this page

Generated deterministically by Sugi Atlas from primary biomedical databases via BioBTree. Every record on this page traces to a primary source.

Generated
Atlas version
v1.1.4
BioBTree
v2.0.2

Sources 25

This page pulls from 25 datasets indexed by BioBTree:

chembl_moleculecivic_evidenceclinical_trialsclinvardoidefogardgenccgwasgwas_studyhgncicd10cmicd11meddramedgenmeshmimmondomondochildmondoparentncitnordorphanetsctidumls