Castleman Disease

Overview

What is Castleman disease?

Castleman disease, or Castleman syndrome, refers to a group of rare disorders involving an overgrowth of cells in the body’s lymphatic system.

The lymphatic system is made up of lymph nodes (also called lymph glands) and other lymphatic tissue. This system is part of the immune system and filters harmful substances such as bacteria and viruses so they don’t spread to other parts of the body.

Castleman disease can affect one or more lymph nodes in a single region of the body or it can involve multiple lymph node regions. Doctors classify the disease into different categories based on the number of lymph node regions affected.

Castleman disease and its symptoms are similar to lymphomas, cancers that affect the lymph nodes.

How common is Castleman disease?

Castleman disease is rare. Doctors diagnose about 6,500-7,700 new cases in the U.S. each year.

What are different types of Castleman disease?

  • Unicentric Castleman disease (UCD): This form affects a single or multiple lymph nodes in one region of the body. It is also called localized Castleman disease. The cause of UCS remains unclear.
  • Multicentric Castleman disease (MCD): In this form, multiple lymph node regions in the body are involved. Approximately half of MCD cases are caused by HHV-8 infection in people with human immunodeficiency virus (HIV) or otherwise immunocompromised for other reasons. The remaining half of MCD cases are HHV-8 negative and are referred to as HHV-8 negative MCD or idiopathic MCD or iMCD. iMCD can be further classified intro three distinct clinical groups:
    • iMCD associated with POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder and Skin changes)
    • iMCD associated with TAFRO (Thrombocytopenia, Anasarca, Myelofibrosis, Renal dysfunction and Organomegaly)
    • iMCD, not otherwise specified (iMCD-NOS)

Symptoms and Causes

What causes Castleman disease?

Infection such as human herpes virus 8 (HHV-8) and possibly others as well as problems with the body’s immune system may cause Castleman disease. Castleman disease can be associated with other cancers such as lymphoma.

What are the symptoms of Castleman disease?

Signs and symptoms of Castleman disease vary depending on the type. People with unicentric Castleman disease (UCD) do not always have symptoms. Doctors usually discover the disease during an exam for another condition. When symptoms do occur, they include:

  • Pressure or full feeling in the abdomen (belly) or chest
  • Lump beneath the skin in the armpit, neck, or groin
  • Unexplained weight loss

Signs of multicentric Castleman disease (MCD) include:

  • Fever
  • Night sweats
  • Fatigue (extreme tiredness)
  • Appetite and weight loss
  • Abnormally large lymph nodes, typically in the neck, armpit, collarbone, and groin
  • Enlarged spleen or liver
  • Anemia (low amount of red blood cells)

Diagnosis and Tests

How is Castleman disease diagnosed?

The symptoms of Castleman disease are similar to other conditions, including common illnesses such as influenza (the flu). A doctor uses several tests to rule out these conditions and confirm a diagnosis of Castleman disease. These tests include:

  • Biopsy: A doctor takes a sample of tissue from a lymph node and looks at it under a microscope to identify signs of Castleman disease.
  • Blood and urine tests: A doctor takes a sample of blood or urine to evaluate the levels of substances in the body that may be signs of the disease.
  • Imaging tests: Tests such as X-rays and CT scans allow doctors to locate enlarged lymph nodes in the body.

Management and Treatment

How is Castleman disease managed or treated?

Treatment for Castleman disease varies according to the type: unicentric Castleman disease (UCD) or multicentric Castleman disease (MCD). Doctors usually recommend surgery for UCD. This surgery removes the affected lymph nodes.

Depending on the region involved some people receive radiation or immunotherapy before surgery for UCD. These therapies can shrink the tumor to make it easier to remove. Doctors also might recommend radiation or immunotherapy to destroy any part of the tumor that was not removed in surgery.

MCD is more difficult to treat than UCD. Because the multicentric type is widespread, doctors do not typically use surgery or radiation to treat it. Instead, they use:

  • Corticosteroids: Medications that reduce inflammation (swelling)
  • Chemotherapy: Anti-cancer medications that slow the overgrowth of cells in the lymphatic system
  • Immunotherapy: Human-made antibodies (proteins that help fight infections) that boost the immune system. In 2014, siltuximab (Sylvant ®) became the only iMCD therapy approved for use by the FDA.

What complications are associated with Castleman disease?

People with Castleman disease have an increased risk of developing cancers including lymphoma (cancer of the lymph system) and Kaposi’s sarcoma (a cancerous skin tumor).

Some people with MCD develop infections that can damage organs and be life-threatening if they are not treated.

Prevention

What are the risk factors for Castleman disease?

People who have human immunodeficiency virus (HIV) are at higher risk for Castleman disease. There are no other known risk factors.

Can Castleman disease be prevented?

You can reduce your risk of Castleman disease by reducing your risk of being infected with HIV. To prevent the spread of this infection you can:

  • Use condoms during sexual activity
  • Limit the number of your sexual partners
  • Avoid drug use
  • Avoid sharing needles during intravenous (into the vein) drug use

Outlook / Prognosis

What is the prognosis (outlook) for people with Castleman disease?

The outlook is very good for most people with unicentric Castleman disease (UCD) who have the affected lymph node removed. Surgery is typically considered curative. When treated, this condition does not usually affect life expectancy.

The prognosis for people with multicentric Castleman disease (MCD) varies. Some people need ongoing treatments because the disease never goes away fully. In these cases, doctors determine a treatment plan that may include chemotherapy and other therapies to keep the condition from getting worse for as long as possible.

Living With

When should I call the doctor?

Contact your doctor if you find a lump in your neck, armpit or groin, or experience other symptoms of Castleman disease that do not go away after a few weeks.

What questions should I ask my doctor?

If you have Castleman disease, you may want to ask your doctor:

  • Which type of Castleman disease have I been diagnosed with?
  • What kinds of tests should I have?
  • What is the best treatment for me?

When can I go back to my regular activities?

Treatments for Castleman disease vary and have different effects on your ability to perform daily activities. Your doctor will tell you when you can return to your usual schedule.

Resources

Are there organizations that offer support to people with Castleman disease?

You may find additional information and or support from the following organization.

Last reviewed by a Cleveland Clinic medical professional on 03/06/2019.

References

  • American Cancer Society. Accessed 3/7/2019.What is Castleman Disease? (https://www.cancer.org/cancer/castleman-disease/about/what-is-castleman-disease.html)
  • Castleman Disease Collaborative Network. Accessed 3/7/2019. About Castleman Disease. (https://www.cdcn.org/about-castleman-disease)
  • Genetic and Rare Diseases Information Center. Accessed 3/7/2019.Multicentric Castleman Disease. (https://rarediseases.info.nih.gov/diseases/9644/multicentric-castleman-disease)
  • National Organization for Rare Disorders. Accessed 3/7/2019.Castleman Disease. (https://rarediseases.org/rare-diseases/castlemans-disease/)
  • U.S. Department of Health and Human Services. Accessed 3/7/2019.The Basics of HIV Prevention. (https://aidsinfo.nih.gov/understanding-hiv-aids/fact-sheets/20/48/the-basics-of-hiv-prevention)

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