Castleman Disease

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Retrieved
2023-08-16
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Overview

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, usually in the chest or abdomen. This form is called unicentric Castleman disease.

Multicentric Castleman disease, or MCD, affects multiple lymph nodes throughout the body. There are 3 types:

  1. HHV-8-associated MCD. This type is associated with human herpes virus type 8, called HHV-8, and human immunodeficiency virus, or HIV.
  2. Idiopathic MCD. The cause of this type is unknown. This is also called HHV-8-negative MCD.
  3. POEMS-associated MCD. This type is associated with another condition called POEMS syndrome. POEMS syndrome is a rare blood disorder that damages your nerves and affects other parts of your body.

Treatment and outlook vary, depending on the type of Castleman disease you have. The type that affects only one lymph node, unicentric Castleman disease, can usually be successfully treated with surgery.

Symptoms

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

Some people with unicentric Castleman disease might have signs and symptoms that are more commonly seen in multicentric Castleman disease, which may include:

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

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

When to see a doctor

If you notice an enlarged lymph node on the side of your neck or in your underarm, collarbone or groin area, talk to your health care provider. Also call your care team if you have a persistent feeling of fullness in your chest or abdomen, fever, fatigue, or unexplained weight loss.

Causes

It's not clear what causes Castleman disease. However, infection by a virus called human herpes virus 8, or HHV-8, is associated with the HHV-8 version of multicentric Castleman disease.

Studies have found that HHV-8 is present in nearly all HIV-positive people who have Castleman disease and in fewer than of half of HIV-negative people with Castleman disease.

Risk factors

Castleman disease can affect people of any age. But the average age of people diagnosed with unicentric Castleman disease is 35. Most people with the multicentric form are in their 50s and 60s. The multicentric form is also slightly more common in men than in women.

The risk of developing multicentric Castleman disease is higher in people who are infected with HIV.

Complications

People with unicentric Castleman disease usually do well once the affected lymph node is removed. Multicentric Castleman disease may lead to life-threatening infections or organ failure. People who also have HIV/AIDS generally have the worst outcomes.

Diagnosis

After reviewing your medical history and doing a detailed physical exam, your health care provider may recommend:

  • Blood and urine tests, to help rule out other infections or diseases. These tests also can reveal anemia and abnormalities in blood proteins that are sometimes typical of Castleman disease.
  • Imaging tests, to look for enlarged lymph nodes, liver or spleen. A CT scan of your neck, chest, abdomen and pelvis may be used. Positron emission tomography, also known as PET scans, may be used to diagnose Castleman disease and to assess whether a treatment is effective.
  • Lymph node biopsy, to distinguish Castleman disease from other types of lymphatic tissue disorders, such as lymphoma. A tissue sample from an enlarged lymph node is removed and examined in the laboratory.

Treatment

Treatment depends on the type of Castleman disease you have.

Unicentric Castleman disease

Unicentric Castleman disease can be cured by surgically removing the diseased lymph node. If the lymph node is in your chest or abdomen — which is often the case — major surgery may be needed.

If surgical removal isn't possible, medicine may be used to shrink the lymph node. Radiation therapy also may be an effective way to destroy the affected tissue.

You'll need follow-up exams, including imaging, to check for relapse.

Multicentric Castleman disease

Treatment for multicentric Castleman disease generally involves medicines and other therapies to control cell overgrowth. Specific treatment depends on the extent of your disease and on whether you have HIV or HHV-8 infection or both.

Treatment options for multicentric Castleman disease may include:

  • Immunotherapy. The use of drugs such as siltuximab (Sylvant) or rituximab (Rituxan) can block the action of a protein that is made in excess in people who have multicentric Castleman disease.
  • Chemotherapy. This type of medicine can slow the overgrowth of lymphatic cells. Your provider may recommend adding chemotherapy if the disease doesn't respond to immunotherapy or if you have organ failure.
  • Corticosteroids. Drugs such as prednisone can help control inflammation.
  • Antiviral drugs. These drugs can block the activity of HHV-8 or HIV if you have one or both of those viruses.

Preparing for your appointment

You may be referred to a provider who is trained in treating blood disorders called a hematologist.

What you can do

  • Write down the symptoms you have been experiencing and for how long.
  • Write down key medical information, including other health problems.
  • Make a list of all medicines, vitamins and supplements that you're taking.

Questions to ask your doctor:

  • What's the most likely cause of my signs and symptoms?
  • What kinds of tests do I need? Do they require any special preparation?
  • What treatment do you recommend? Do I need surgery?

In addition to the questions that you've prepared to ask your health care provider, don't hesitate to ask other questions during your appointment.

What to expect from your doctor

Your health care team is likely to ask you a number of questions. Being ready to answer them may leave time to go over points you want to spend more time on. You may be asked:

  • Do you have any other health conditions, such as HIV/AIDS or Kaposi's sarcoma?
  • When did you first begin experiencing symptoms?
  • Have your symptoms been continuous or occasional?
  • How severe are your symptoms?
  • Does anything seem to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?