Overview

Castleman disease is a rare disorder that involves an overgrowth (proliferation) of cells in your body's disease-fighting network (lymphatic system). Also known as giant lymph node hyperplasia and angiofollicular lymph node hyperplasia, Castleman disease can occur in a localized (unicentric) or widespread (multicentric) form.

Treatment and outlook vary, depending on the type of Castleman disease you have. The localized type can usually be successfully treated with surgery.

Sometimes associated with HIV infection, multicentric Castleman disease can be life-threatening. Multicentric Castleman disease is also associated with other cell-proliferation disorders, including cancer of the lymphatic system (lymphoma), Kaposi's sarcoma and POEMS syndrome.

Symptoms

There are two basic types of Castleman disease:

  • Unicentric Castleman disease. This localized form of the disease affects only a single gland (lymph node) in your lymphatic system.
  • Multicentric Castleman disease. This type affects multiple lymph nodes and lymphatic tissues, and can severely weaken your immune system.

Multicentric Castleman disease can be further classified as:

  • Multicentric Castleman disease without POEMS syndrome
  • Multicentric Castleman disease with POEMS syndrome that involves areas of abnormal bone (osteosclerotic lesions)
  • Multicentric Castleman disease with POEMS syndrome without osteosclerotic lesions

Unicentric Castleman disease

Many people with unicentric Castleman disease don't notice any signs or symptoms. The diseased lymph node is usually located in the chest, neck or abdomen. When signs and symptoms are present, they may include:

  • A feeling of fullness or pressure in the chest or abdomen that can cause difficulty breathing or eating
  • An enlarged lump under the skin in the neck, groin or armpit
  • Unintended weight loss
  • Less commonly, fever, night sweats and weakness

Multicentric Castleman disease

Most people with multicentric Castleman disease experience:

  • Fever
  • Night sweats
  • Fatigue and weakness
  • Loss of appetite
  • Unintended weight loss
  • Enlarged lymph nodes, usually around the neck, collarbone, underarm and groin areas
  • Enlarged liver or spleen

Other, less common symptoms include:

  • Nerve damage in the hands and feet that leads to numbness (peripheral neuropathy)
  • Skin rash

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 doctor. Also call your doctor if you experience 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 herpesvirus 8 (HHV-8) is associated with multicentric Castleman disease.

The HHV-8 virus has also been linked to the development of Kaposi's sarcoma, a cancerous tumor of the blood vessel walls that can be a complication of HIV/AIDS. Studies have found that HHV-8 is present in HIV-positive people who have Castleman disease, and in 40 to 50 percent of HIV-negative people with Castleman disease.

The precise role of HHV-8 is unclear. But it appears to cause malfunctioning immune system cells to reproduce rapidly. The immune system cells produce a protein called interleukin-6 (IL-6) that contributes to the overgrowth of lymphatic cells.

Risk factors

Castleman disease can affect anyone. 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 only known risk factor for Castleman disease appears to be having HIV/AIDS.

Complications

People with unicentric Castleman disease usually do well once the affected lymph node is removed. However, having Castleman disease may increase your risk of lymphoma.

Complications of multicentric Castleman disease can be life-threatening and may include:

  • Infection leading to the failure of multiple organs
  • Cancer, such as lymphoma or Kaposi's sarcoma

The outlook for people with multicentric Castleman disease varies, depending on the nature of their disease. The presence of HIV/AIDS tends to worsen the outcome.

Research also indicates that people who have multicentric Castleman disease with POEMS syndrome that doesn't involve bone lesions may have worse outcomes, while people who have multicentric Castleman disease with the bone lesion variant of POEMS syndrome tend to do better.

Aug. 27, 2014
References
  1. AskMayoExpert. What are the symptoms of Castleman disease? Rochester, Minn.: Mayo Foundation for Medical Education and Research; 2014.
  2. Brown JR, et al. Unicentric Castleman's disease. http://www.uptodate.com/home. Accessed July 14, 2014.
  3. Aster JC, et al. Multicentric Castleman's disease. http://www.uptodate.com/home. Accessed July 14, 2014.
  4. Dispenzieri A, et al. The clinical spectrum of Castleman's disease. American Journal of Hematology. 2012;87:997.
  5. Fajgenbaum DC, et al. HHV-8-negative, idiopathic multicentric Castleman disease: Novel insights into biology, pathogenesis, and therapy. Blood. 2014;123:2924.
  6. Robinson D, et al. Clinical epidemiology and treatment patterns of patients with multicentric Castleman disease: Results from two US treatment centers. British Journal of Haematology. 2014;165:39.
  7. Talat N, et al. Surgery in Castleman's disease: A systematic review of 404 published cases. Annals of Surgery. 2012;255:677.