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)

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)

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