This condition is infrequent and manifest as overgrown cells in the lymph nodes. Most commonly it presents as a single node enlargement in chest or abdomen known as unicentric castleman disease whereas multiple involvement of node is known as multicentric castleman disease.
This condition presents as
- Unicentric condition mostly don’t show signs or symptoms
- Multicentric lymph nodes enlargement are presented with high grade fever
- Unexpected weight loss
- Sweating at night
- Spleen or liver enlargement
- Location of most of the multicentric lymph nodes are groin areas,neck or collar bone
Major cause of lymph nodes enlargement is not exactly known but many studies shows there is a link between human herpes virus-8 (HHV-8) and multicentric lymph nodes. This virus is also linked to the development of special type of skin cancer in AIDs patient known as Kaposi’s sarcoma. Some research data shows that castleman diseases patients who are HIV-positive have HHV-8 mostly unlikely is the case with HIV-negative patients.
- Multicentric castleman disease can sometimes lead to the complicated life-threatening condition .
- Major organ failure can also result from long term condition.
- This condition lead patient prone to different types of lymphomas.
Firstly a detailed family history and complete and thorough general physical examination is needed to have good diagnosis. Other tests include;
- Blood test and urine test detect different type of infectious agents which could be the cause of this condition. Moreover, it also helps to see the blood count and condition which could be helpful in diagnosing castleman syndrome.
- CT scan, MRI or PET is done as imaging test to screen lymph nodes, liver and spleen.
- Biopsy of lymph nodes is sometimes done to rule out any other type of lymphomas.
Treatment options include
- For unicentric castleman syndrome surgical removal of lymph node is done however, most of the time it is located inin chest or abdomen major surgery is the only option. Before surgery size of large lymph node is shrinked so that it’s easy to excise.
- For multicentric lymph nodes medication are preferred in order to control the overgrowing cells for example; corticosteroids, anti viral drugs ( in case patient have HIV or HHV-8 infection).
- Sometimes immunotherapy is considered for multicentric castleman disease in which some drugs are used to stop the protein formation which is excessively producing in body. Drugs include; situximab or rituximab.