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.
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.
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.
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:
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)
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.
Tests such as X-rays and CT scans allow doctors to locate enlarged lymph nodes in the body.
Medications that reduce inflammation (swelling)
Anti-cancer medications that slow the overgrowth of cells in the lymphatic system
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.
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.