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Lymph nodes in the human bodyCastleman disease (CD) is a rare disease that affects your lymph nodes.

Lymph nodes are part of your immune system. They contain specific types of cells which help your body fight off infections. Groups of lymph nodes are found in lots of different parts of your body.

In Castleman Disease, the number of cells in a lymph node increase – the cells multiply more than normal. This causes the lymph node to get bigger. It also causes small changes in the lymph node that can only been seen using a microscope.

It is important to know that Castleman disease is not a cancer, however it can increase the risk of developing some types of cancer.

Anyone can develop Castleman Disease – men, women, adults or children.

Sources:
American Cancer Society: https://www.cancer.org/cancer/castleman-disease
Genetic and Rare Diseases Information Centre, Castleman disease: https://rarediseases.info.nih.gov/diseases/12656/castleman-disease
National Organization for Rare Disorders: https://rarediseases.org/rare-diseases/castlemans-disease//


What is Castleman disease?

There are two main types of Castleman disease – unicentric and multicentric.


Unicentric Castleman disease

Castleman diseaseWhat is UCD?
In Unicentric Castleman disease (UCD), one group of lymph nodes has one or more lymph nodes that are larger than normal and have microscopic changes. The affected lymph nodes are usually in the chest or stomach area.

What causes UCD?
We do not know the exact cause of UCD. However many doctors and scientists believe it is caused by changes in the immune system.

What is the prognosis (long-term outlook) for people with UCD?
Many patients can be cured and, if successfully treated, UCD does not affect life-expectancy. Over 90% of patients are alive 5 years after diagnosis.1

How is UCD treated?
UCD is usually treated by having an operation to remove the affected lymph node. Additional treatments, such as radiotherapy or medications, may be used if the operation is not possible or successful.

References: :
1. Zhang X, Rao H, Xu X, et al. Clinical characteristics and outcomes of Castleman disease: A multicenter study of 185 Chinese patients. Cancer Sci. 2018; 109(1): 199–206

Sources:
American Cancer Society: https://www.cancer.org/cancer/castleman-disease
Genetic and Rare Diseases Information Centre, Unicentric Castleman disease: https://rarediseases.info.nih.gov/diseases/6005/unicentric-castleman-disease
National Organization for Rare Disorders: https://rarediseases.org/rare-diseases/castlemans-disease//

Multicentric Castleman disease

Multicentric Castleman diseaseWhat is MCD?
In Multicentric Castleman disease (MCD), two or more groups of lymph nodes contain lymph nodes that are larger than normal and have microscopic changes.

What causes MCD?
MCD can be caused by a virus called human herpesvirus 8. This type of MCD is usually found in people with weakened or poor immune systems. However in many patients, the cause of MCD is not known. This type of MCD is called idiopathic MCD.

What is the prognosis (long-term outlook) for people with MCD?
MCD is a very serious disease and can be fatal. Data indicate that 65% of patients with idiopathic MCD are alive 5 years after diagnosis.1

How is MCD treated?
Treatment of MCD usually involves drugs that target specific parts of your immune system. Your doctor will recommend a treatment depending on the type of MCD.

References: :
1. Fajgenbaum DC et al. International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. Blood 2017; 129(12): 1646-1657

Sources:
American Cancer Society: https://www.cancer.org/cancer/castleman-disease
Genetic and Rare Diseases Information Centre, Multicentric Castleman disease: https://rarediseases.info.nih.gov/diseases/9644/multicentric-castleman-disease
National Organization for Rare Disorders: https://rarediseases.org/rare-diseases/castlemans-disease/