Castleman disease


[Benjamin Castleman, U.S. pathologist, 1906-1982]
ABBR: CD An occasionally aggressive illness marked by excessive growth of lymphoid tissue either localized in a single lymph node group (unicentric) or in multiple regions of the body (multicentric).

The disease is rare; it occurs in about 1 in 1,000,000 people.

Although the cause is not precisely known, its associations with HIV/AIDS, Kaposi sarcoma, and human herpesvirus 8 infection have led some to propose that it has an infectious basis.

Many people experience no symptoms for unicentric CD; however, patients with a very large mass may experience: a feeling of fullness in the upper abdominal area; a lump under the skin in the lymph node area (neck, armpit, or groin); weight loss; cough; or anemia. For multicentric CD, symptoms usually include fever, night sweats, loss of appetite, weight loss, weakness, fatigue, enlarged lymph nodes, enlarged liver or spleen, and/or nerve damage in the hands or feet.

Biopsy of an enlarged lymph node.

Localized disease responds well to surgical resection. Widespread disease can sometimes be treated effectively with chemotherapy, corticosteroids or immunotherapy. CD patients with HIV have a more difficult time eradicating the disease than non-HIV infected patients because of the other diseases that are usually associated with HIV.

The health care team supports the patient medically and psychologically by providing education about the disease and its treatment, answering questions, providing comfort, and alleviating anxiety.

SYN: SEE: angiofollicular lymph node hyperplasia