Castleman Disease

What is Castleman Disease?

Castleman disease is a disorder that is considered rare. This disorder involves the body’s defense system and disease fighting network creating cells in an out of control manner. The disease fighting network involved in this process is the lymphatic system. This disorder is also referred to by medical professionals as lymph node hyperplasia or angiofollicular lymph node hyperplasia.

This disease can be considered localized or it can be considered widespread. The treatment offered by your doctor will depend on the form of the disease you have. The outlook for the condition also depends on this. Localized Castleman disease can usually be treated with surgery successfully.

Sometimes, this disease is associated with being a secondary condition with an existing HIV infection. A diagnosis of multicentric Castleman disease may be considered life threatening.  This disorder has also been associated with other diseases that fall into the cell-proliferation disorder, including lymphoma, POEMS syndrome, and Kaposi’s sarcoma.

What are the Symptoms of Castleman Disease?

There are two main types of Castleman disease, unicentric Castleman disease and Multicentric Castleman disease.

Unicentric Castleman Disease

This is the localized form of the disease. It only affects one gland of the lymphatic system. Patients with unicentric Castleman disease usually do not have any symptoms. Typically, the affected lymph node is located in the abdomen, chest or neck.

Unicentric symptoms include

  • Pressure or fullness in the area of the affected lymph node. If the lymph node is in the chest or neck, it can make it difficult to eat or breathe.
  • A large lump under the skin in the affected area.
  • Unexplained weight loss.
  • Fever
  • Night sweats
  • Weakness without explanation

Multicentric Castleman Disease

Multicentric symptoms include

This widespread form of the disease can affect any lymph node or lymphatic tissue in the system. It can extremely weaken the patient’s immune system.

  • Unexplained fever
  • Night sweats
  • Unexplained fatigue
  • Unexplained weakness
  • Appetite changes
  • Unexplained weight loss
  • Swollen lymph nodes in the affected areas
  • Enlarged spleen
  • Enlarged liver

Less common symptoms

Some patients experience symptoms that are less common.

  • Symptoms of nerve damage in the hands and feet
  • Numbness of the hands and feet
  • Rash on the skin

Castleman Disease Causes

Castleman Disease (CD) is also known as giant lymph node hyperplasia or angiofollicular lymph node hyperplasia. Technically, CD is not a cancerous condition, but is related to lymphoma. It is considered a lymphoproliferative disorder, so while it is not malignant, CD does exhibit many of the same behaviors as a lymphoma and can be quite serious for the patient.

There are two major forms of CD. Unicentric CD is the most common and only affects one group of lymph nodes and is generally less serious. It can often be treated through surgical removal. The more serious form of CD is Multicentric CD (known as MCD), which affects more than one group of lymph nodes at a time. This form is rare and occurs most frequently in patients with human immunodeficiency virus (HIV), where the patient’s immune system is already impacted. Scientists are not sure what directly causes any form of CD, but the virus HHV-8 has been directly linked to MCD in patients with HIV/AIDS. Current research indicates 4 possible causes of Castleman Disease. They are exposure to certain viruses or genetic mutations that occur throughout the patient’s life, chronic inflammation, inherited mutations and autoimmunity. CD affects men and women equally.

How is Castleman Disease Treated?

Treatment for unicentric Castleman disease usually involves surgery to remove the affected lymph node. This surgery is considered major surgery, since the most common location for this disease to affect is in the chest and abdomen.

Some patients may require radiation therapy before or after surgery to help kill off any remaining abnormal cells.

Multicentric Castleman disease is more difficult to treat. Surgery is typically not an option for this type of Castleman disease. This is because of the number of lymph nodes that are involved and the danger involved in removing this many.

The treatment chosen will depend on the severity of the disease and whether or not HIV or HHV-8 are present. Multicentric Castleman disease does have a lot of options for treatment. These treatments can include monoclonoal antibodies, chemotherapy, corticosteroids, antiviral drugs, and thalidomide.

Castleman Disease Prevention

In terms of prevention, the one known cause of MCD is the virus HHV-8, which is associated with HIV/AIDS, therefore one method of prevention would be avoiding those behaviors that can lead to acquisition of HIV/AIDS. Abstinence is the surest form, but protected sex with consistent use of latex condoms, and other behaviors associated with safe sex can prevent exposure to the virus directly linked to MCD. In terms of some of the other suspected causes, they are difficult to prevent as exposure to unknown viruses or genetic mutations is next to impossible. As always, a healthy diet and a generally healthy lifestyle are the best preventions of any illness.