Castleman Disease Collaborative Network
Targeted Grantee
Castleman disease (CD) describes a group of rare disorders that involve enlarged lymph nodes and a broad range of inflammatory symptoms and laboratory abnormalities. The lymph nodes, and the cells that reside in them (lymphocytes and stromal cells), are an integral part of our immune system that help us fight invading organisms and cancer. In a healthy individual, the cells of the immune system become activated to fight off invading organisms or cancer and then return to a surveillance mode. In CD, the cells of the immune system become hyperactivated, overproduce cytokines and other inflammatory compounds, and fail to return to a surveillance mode. Approximately 4,300-5,200 cases of CD are diagnosed in the US each year; CD can occur in people of any age, gender, or ethnicity. All forms of CD involve a constellation of microscopic abnormalities in the lymph node tissue that can be observed following a lymph node biopsy. Whether Castleman disease should be considered an autoimmune disease, cancer, or infectious disease is currently unknown. The symptoms, causes, and treatments vary greatly for each subtype of CD.
Last updated 04/30/2026
Clinical
Disease Class
Hematological diseases
Immunological diseases
Infectious diseases
Neoplastic diseases
Systemic and rheumatological diseases
Body Systems
Hematopoietic / Lymphatic / Immune
Organs
Blood
Bone marrow
Kidneys
Liver
Lymph fluid, nodes, ducts, vessels
Spleen
Known Genetic Link
No, the disease is not known to have a genetic basis
Newborn Screening
No
Disease Mechanism(s)
Aberrant immune response
Abnormal cell proliferation
Autoimmune Disease
Cell signaling defects
Vascular/Angiogenesis Defects
mTOR pathways dysregulation
Age of Onset
Adolescence (12-17)
Adulthood (age 18-64)
Early childhood (age 1+-5)
Elderly (age 65+)
Middle childhood (6-11)
Average Age at Diagnosis
Adolescence (12-17)
Adulthood (age 18-64)
Elderly (age 65+)
Life Expectancy
Adulthood (age 18-64)
Elderly (age 65+)
Affected Sex(es)
Female
Male
National Prevalence
Unknown
Global Prevalence
Unknown
National Incidence
Less than 10
Global Incidence
Less than 10
Populations and/or ancestry with higher prevalence
HHV-8–associated disease burden is higher in regions/populations with higher HHV-8 prevalence
Symptoms / Phenotypes
anemia
edema
elevated CRP
fatigue
fever
hepatosplenomegaly
hypoalbuminemia
inflammation
inflammatory abnormality of the skin
night sweats
organ failure
peripheral neuropathy
swollen lymph nodes
weight loss
Biomarkers
Diagnostic
· Lymph node histopathology on biopsy; HHV-8 testing for HHV-8–associated MCD
Monitoring
· CRP/ESR, CBC (anemia/cytopenias), albumin, renal function; IL-6 pathway activity
Prognostic
· Subtype classification (UCD vs MCD; HHV-8 status), severity features (e.g., organ dysfunction)
Therapeutic
· Markers reflecting response to IL-6–targeted therapy
Existing Therapies
Drugs used off-label
Other
· Surgical resection is standard/curative intent for unicentric Castleman disease
Regulatory Agency-Approved to Cure or Modify the Disease
· Siltuximab (SYLVANT) for HIV-negative, HHV-8-negative multicentric Castleman disease
Therapies in Development
Antibody-based therapy (monoclonal antibodies, biologics)
· 2; Siltuximab, Tocilizumab
Immunotherapy
· 2; Rituximab, IL-6 pathway–targeted therapies
Other (please specify)
· 1; Chemotherapy-based regimens
Repurposed drug
· 4; Rituximab, Sirolimus, Tocilizumab, Corticosteroids
Small molecule therapy (novel small molecule drugs)
· 1; Sirolimus
Surgical & interventional
· 1; Surgical Intervention
Therapeutic Development Stages
Approved/Available
In clinical trials (Phase I, II, III, or IV)
In preclinical development
In research/exploratory phase
Therapeutic Development Role
Access to registry or natural history study
Data sharing
Outcome measures development
Recruitment and outreach to patients
Recruitment and outreach to trial sites / physicians
Results dissemination (including publications)
Organizational & Research
Cell Lines
None
Disease Model
Mouse
Disease Model, Involvement
Funded
Disease Model, share
No
Organizational Challenges
Not at this time
Clinical Trial Role
Data sharing
Funding
Outcome measures, development
Recruitment and outreach, patients
Recruitment and outreach, trial sites/physicians
Results dissemination, publication
Study material design, review (not protocol)
Travel coordination
Clinical Trial Types
Phase 1
Biobank, Institution
University of Pennsylvania (PENN)
Biobank, Involvement
Designed
Funded
Registry
Yes, we have a registry that we created
Data Collected, Registry
Clinical data
Electronic health records/electronic medical records
Imaging data
Longitudinal natural history data
Medication usage
Patient contact info
Patient-reported data
Data Entered by, Registry
Other
Platform, Registry
Pulse InfoFrame
Natural History Study
Yes, we have a natural history study that we created
Data Collected, Natural History Study
Clinical endpoints (outcomes)
Electronic health records/electronic medical records
Imaging data
Medication usage
Patient-reported outcomes
Prospective data
Platform, Natural History Study
Pulse InfoFrame
FDA Patient Listening Session
No
FDA Patient-Focused Drug Development (PFDD) Program
No
ICD Codes
Yes, we have an ICD-10 code specific to our exact disease
Diagnostic Guidelines
Yes, we have published formal guidelines in a peer-reviewed journal
Science Advisory Board Policies
No policies
Research Network Policies
Has CRN and willing to share policies
Patient Priority Survey
No
Research Roadmap
Yes we have a Research Roadmap, and will share policies
International Chapters
None
International Partners
Africa
Asia
Europe
Other International Research Initiatives
Asia
Europe
North America