Castleman Disease: Causes, Symptoms, Treatment

What is Castleman Disease?

Castleman Disease is a relatively uncommon disorder of the lymphatic system in which there is an abnormal growth of cells. Castleman Disease is also known by the name of Giant Lymph Node Hyperplasia. There are basically two types of this disease which are the unicentric and the multicentric. The treatment of Castleman Disease depends on the type of the disease the individual has and the severity of it. Surgery is usually the preferred form of treatment. If Castleman Disease occurs individuals already suffering from HIV infection then this disease may take a turn towards the worse and can become lethal, especially in the multicentric form of this disease. Castleman Disease can also occur in cases when an individual is suffering from a cancerous situation like a lymphoma.

What is Castleman Disease?

What Causes Castleman Disease?

The root cause as to what causes Castleman disease is still a mystery, although some studies suggest that it is caused by a herpes virus knows as HHV-8. This HHV-8 virus is also known to be a complication of AIDS. Studies suggest that the virus HHV-8 is found more in people who suffer from HIV/AIDS than who do not have it.

What are the Risk Factors for Castleman Disease?

There is no specific risk factor for Castleman Disease. It can affect anyone but generally it strikes people who are above 35 years of age. The multicentric form of this disease affects people who are more than 50 years of age. Multicentric form of Castleman Disease is found more in males than females. The only apparent risk factor for Castleman Disease as of now is any individual having HIV/AIDS.

What are the Symptoms of Castleman Disease?

The symptoms of Castleman Disease depend on the type of the disease the patient has. As stated, there are two forms of Castleman Disease which is the unicentric and the multicentric type. In unicentric type of Castleman Disease, only one lymph node gets affected where as in the multicentric type multiple lymph nodes are affected. Some of the symptoms experienced by people affected by Castleman Disease are:

  • Chest pressure or discomfort causing difficulty with eating or swallowing
  • Presence of a lump in the groin, neck, or armpit
  • Unintentional loss of weight
  • In some cases fever and weakness
  • Easy fatigue
  • Appetite loss or extremely poor appetite
  • Lymph node enlargement
  • Hepatosplenomegaly
  • In some cases, peripheral neuropathy
  • Skin rashes.

How is Castleman Disease Diagnosed?

In most of the cases of Castleman Disease, the diagnosis is made incidentally when tests are being conducted to rule out some other condition. In case if Castleman Disease of any type is suspected then the following tests may be ordered to confirm the diagnosis.

Blood Tests: This test will be done to find if there is an infectious process going on in the body and also to see if there is presence of any anemia causing the symptoms. This test at times identifies abnormalities in the blood protein which is a characteristic feature of Castleman Disease.

MRI/CT Scans: imaging tests are quite a useful tool in identifying Castleman Diseases it can easily identify any abnormal enlargement of the lymph nodes and can help in conforming the diagnosis of Castleman Disease.

Lymph Node Biopsy: This can be done to rule out other disease and differentiate Castleman Disease from other diseases.

How is Castleman Disease Treated?

Treatment of Castleman Disease depends on the type of disease the patient has. In cases of Unicentric Castleman Disease, since only one lymph node is involved it can be surgically corrected by removing the diseased lymph node. In case of the disease lymph node is located in a position where it cannot be surgically removed, then medications may be given for shrinking the diseased lymph node and then eliminate it. This is best done using radiation therapy. Since relapses are common in this form of Castleman Disease hence frequent followups and imaging studies are recommended.

When it comes to Multicentric form of Castleman disease surgery is not the treatment of choice as there are multiple lymph nodes that are involved, although it is seen that a splenectomy is quite effective in eradicating most of the diseased lymph nodes. In such cases, medication and alternative therapies are used for controlling the symptoms but again it depends upon whether you just have Castleman Disease or is it in combination with HIV/AIDS which is often the case. Some of the options for treatment for Castleman Disease are:

  • Monoclonal Antibodies: These can be given to block the protein which promotes cell overgrowth.
  • Chemotherapy: This therapy may be given to slow down the overgrowth of cells.
  • Corticosteroids: This will be given to control inflammation.
  • Antiviral drugs: This will be given to keep a check on viral overload in cases of HIV/AIDS and control the symptoms of Castleman Disease.
Pramod Kerkar, M.D., FFARCSI, DA
Pramod Kerkar, M.D., FFARCSI, DA
Written, Edited or Reviewed By: Pramod Kerkar, M.D., FFARCSI, DA Pain Assist Inc. This article does not provide medical advice. See disclaimer
Last Modified On:April 10, 2019

Recent Posts

Related Posts