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.

Overeview #

• 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.

Causes #

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.

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 #

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.

Treatment #

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.

Prognosis #

• 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.