Symptoms
Three types of Castleman disease have been identified. These are hyaline-vascular type (about 90% of individuals), plasma cell type, and multicentric or generalized (MCD). All people with Castleman disease develop one or more tumors in lymph node tissue in the body. The symptoms a person with Castleman disease experiences depend upon which type of the disease he or she has.
- Hyaline-vascular type: Tumors develop in the chest, stomach, and/or neck, and symptoms are related to the size and location of the tumor. Many individuals (58-97%) have no symptoms. About 70% of individuals with this type are less than 30 years old. If the tumor is in the chest, there may be a dry cough, shortness of breath, or frequent respiratory infection.
- Plasma cell type: Individuals may have fever, weight loss, fatigue, and skin rash. This type of Castleman disease affects blood cells. It causes destruction of red blood cells, leading to low numbers of red blood cells in the body (called hemolytic anemia), or abnormally high amounts of immune factors in the blood (called hypergammaglobulinemia).
- Multicentric (generalized) type: This type affects many areas of the body. The symptoms may be similar to those of the plasma cell type, and may include enlarged liver and spleen (hepatosplenomegaly). Individuals with multicentric Castleman disease tend to be older, in their 50s or 60s.
Diagnosis
Diagnosis of Castleman disease is based on the symptoms such as the presence of a tumor as well as microscopic examination of the tumor tissue and blood tests.
Treatment
Treatment for Castleman disease focuses on the relieving the symptoms. Treatment for the tumors in the hyaline-vascular type of Castleman disease is surgical removal of the tumor. In most cases the tumor does not grow back. In the plasma cell and multicentric types, corticosteroids or chemotherapeutic drugs may be used, although research has not identified a standard or best treatment.
Castleman disease research
There has been research that has identified interleukin-6, an immune factor, as being responsible for multicentric Castleman disease. Researchers in Japan therefore conducted a study to try an anti-interleukin-6 drug (called MRA) as a treatment for multicentric Castleman disease. They gave the drug to 28 affected individuals, and found that over time MRA relieved symptoms such as fatigue and improved blood test values. This research suggests that an anti-interleukin-6 drug might be a way to treat multicentric Castleman disease.
Sources:
- "Castleman's Disease." Index of Rare Diseases. National Organization for Rare Disorders. 6 Dec 2006 <http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Castleman%27s%20Disease>.
- "Castleman's Variants." Castleman's Disease Homepage. Castleman's Disease Organization. 6 Dec 2006 <http://www.castlemans.org/castleman's_variants.htm>.
- Nishimoto, Norihiro, et al. "Humanized anti-interleukin-6 receptor antibody treatment of multicentric Castleman disease." Blood 106(2005): 2627-2632.
