Castleman Disease
Evidence: Immune-mediated
Names
Castleman Disease
Castleman’s Disease
Angiofollicular lymph node hyperplasia
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:
Unicentric Castleman disease (UCD)
POEMS-associated multicentric Castleman disease (POEMS-MCD)
HHV-8-associated multicentric Castleman disease (HHV-8+MCD)
HHV-8-negative/idiopathic multicentric Castleman disease (iMCD)
Patient Groups
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)
References
Updated November 2023