Castleman Disease

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Castleman disease is a rare disorder that involves an overgrowth of cells in your body’s lymph nodes. The most common form of the disorder affects a single lymph node (unicentric Castleman disease), … Read More

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Castleman Disease

Castleman disease is a rare disorder that involves an overgrowth of cells in your body’s lymph nodes. The most common form of the disorder affects a single lymph node (unicentric Castleman disease), usually in the chest or abdomen. Multicentric Castleman disease affects multiple lymph nodes throughout the body and has been associated with human herpesvirus type 8 (HHV-8) and human immunodeficiency virus (HIV). Treatment and outlook vary, depending on the variety of Castleman disease you have. The type that affects only one lymph node can usually be successfully treated with surgery.  Although Castleman disease is not a form of cancer, it is in many ways similar to lymphomas (cancers of lymph nodes). In fact, many people with this disease eventually end up developing lymphomas and can also be treated with some of the lymphoma treatment options.

Types of Castleman disease

  • Unicentric Castleman Disease: This is the most common form of the disorder, it affects a single lymph node, usually in the chest or the abdomen.
  • Multicentric Castleman Disease: This affects multiple lymph nodes throughout the body. It has been associated with human herpes virus type 8 (HHV-8) and human immunodeficiency virus (HIV).

Castleman disease diagnosis

Castleman disease diagnosis can be made with the following investigations Thorough physical examination.
  • Blood and urine tests: To rule out other infections or diseases, and check for anemia and other abnormalities in blood proteins that are characteristic of Castleman disease.
  • Imaging tests: Such as CT scan, MRI, ultrasound, and PET scan can be recommended to detect enlarged lymph nodes, liver, or spleen.
  • Lymph node biopsy: A sample is taken from an enlarged lymph node’s tissue and examined in the laboratory to differentiate Castleman disease from other kinds of lymphatic tissue disorders, like lymphoma. 

Castleman disease treatment

Castleman disease treatment depends on the type of Castleman disease being treated.
  • Unicentric Castleman Disease
The primary choice of treatment for unicentric Castleman disease is the surgical removal of the diseased lymph node. Alternatively, medication can be used to contract the lymph node. Radiation therapy may also be effective in destroying the affected tissue.
  • Multicentric Castleman disease
As for multicentric Castleman disease treatment, it generally involves medications and other therapies to stem the overgrowth of cells. Specific treatment, however, depends on the extent of the disease and if there’s an HIV and/or HHV-8 infection. Other multicentric Castleman disease treatment options may include;
  • Immunotherapy: The helps block the action of a protein that’s excessively produced in people who have multicentric Castleman disease.
  • Chemotherapy: This helps slowdown the overgrowth of lymphatic cells.
  • Corticosteroids: To help control inflammation.
  • Antiviral drugs: These drugs help block the activities of HHV-8 and/or HIV.

Symptoms

Many people with unicentric Castleman disease don't notice any signs or symptoms. The enlarged lymph node may be detected during a physical exam or an imaging test for some unrelated problem.

Unicentric castleman disease does not present with any specific signs or symptoms. However, an enlarged lymph node may be detected during a physical exam or an imaging test.

For multicentric castleman disease, the following symptoms may be experienced;

  • Fever.
  • Loss of appetite and unintended weight loss.
  • Fatigue and weakness.
  • Night sweats.
  • Vomiting and nausea.
  • Enlarged liver or spleen.
  • Trouble breathing.
  • Wheezing or coughing.
  • Feeling of fullness in the chest and the stomach.
  • Trouble eating.
  • Enlarged lymph nodes.
  • Rashes.
  • Weak or numb hands or feet.

Some people with unicentric Castleman disease might experience signs and symptoms more common to multicentric Castleman disease, which may include:

• Fever
• Unintended weight loss
• Fatigue
• Night sweats
• Nausea
• Enlarged liver or spleen

The enlarged lymph nodes associated with multicentric Castleman disease are most commonly located in the neck, collarbone, underarm and groin areas.

Causes

It's not clear what causes Castleman disease. However, infection by a virus called human herpesvirus 8 (HHV-8) is associated with multicentric Castleman disease.

Several studies have found HHV-8 to be present in nearly all of the persons who have Castleman disease and are HIV positive, and in about half of HIV negative persons with Castleman disease.

Castleman disease might also be associated with problems of immune system, and changes in genes.

The HHV-8 virus has also been linked to the development of Kaposi's sarcoma, a cancerous tumor that can be a complication of HIV/AIDS. Studies have found that HHV-8 is present in nearly all HIV-positive people who have Castleman disease, and in about half of HIV-negative people with Castleman disease.

FAQ

Are there any risk factors for Castleman disease?

The risk of developing Castleman disease can be increased by;

  • Human herpesvirus 8 (HHV-8) infection: This increases the risk of developing multicentric Castleman disease.
  • Sex: Multicentric Castleman disease is slightly more common in men than in women.

What complications does Castleman disease cause?

The following are the complications that may occur from an infection of Castleman disease

  • Life-threatening infections.
  • Organ failure.
  • Cancer; such as lymphoma and Kaposi sarcoma.
  • Dysfunction of immune system.

What is Castleman disease prognosis?

For unicentric Castleman disease, normal routine activities can be resumed once the lymph node is removed, and there probably won't be any more problems afterwards.

As for multicentric Castleman disease, treatment helps stop it from coming back for a long time. This is known as being in remission.

However, for some people, multicentric Castleman disease never goes away completely. Therefore, regular treatment may be required.