Description

Castleman disease (CD) is a group of rare disorders that involve enlarged lymph nodes due to an unexplained activation of the immune system that leads to a hyper-inflammatory state, causing damage to multiple organs. Based on the number of regions in the body that have enlarged lymph nodes, CD can be classified as unicentric or multicentric. This difference is important because unicentric CD and multicentric CD require different treatments. There are four main types of CD:

1. Unicentric Castleman disease (UCD)

2. POEMS-associated multicentric Castleman disease (POEMS-MCD)

3. HHV-8-associated multicentric Castleman disease (HHV-8+MCD)

4. HHV-8-negative/idiopathic multicentric Castleman disease (iMCD)

Patient Groups

Castleman Disease Collaborative Network
MCD Castleman Disease Patient Resource
Facebook Group: Castleman Disease Support Group

Bloggers

None found.

Prevalence

US cases between 1,233 and 4,433 cases [Mukherjee 2022]

Typical Age of onset

Patients of all ages, including young children, can be diagnosed with any form of CD

Symptoms

Symptoms vary depending on the type of CD. Those with unicentric CD may only experience enlarged lymph nodes that are creating pressure on a nearby organ. Other times, enlarged lymph nodes are noticed during imaging for another issue.

Multicentric CD may cause more noticeable symptoms such as:

• Fever

• Night sweats

• Nausea and vomiting

• Unexplained weight loss

• Fatigue (extreme tiredness)

• Abnormally large lymph nodes

• Enlarged spleen (splenomegaly)

• Enlarged liver (hepatomegaly)

• Numbness in your hands and feet

• Swelling in your feet and ankles

• Swelling in your abdomen

• Anemia (low amount of red blood cells)

Resources

Link to Clinical Trials

Link to Scientific Publications

References

ASH Publications
Cleveland Clinic

Profile by Erica Matute-Arcos