Castleman's disease is a rare disease of the lymphatic system. It involves hyperproliferation of certain B cells that often produce cytokines. There are several variants of this disease. One called multicentric Castleman's disease (MCD) is caused by Kaposi's sarcoma-associated herpesvirus (KSHV), a gammaherpesvirus that is also the cause of Kaposi's sarcoma and primary effusion lymphoma. MCD most commonly develops in immunosuppressed patients such as those with AIDS. The most striking clinical features of MCD are high fevers, anemia, and low white blood cell counts, which appear to be due to the overproduction of interleukin 6. There is no standard therapy for Castleman's disease; MCD carries a poor prognosis of about 2 years.

References

• Boshoff C, Weiss R. AIDS-related malignancies. Nat Rev Cancer 2002;2(5):373-82.
• Aoki Y, Yarchoan R, Wyvill K, Okamoto S, Little RF, Tosato G. Detection of viral interleukin-6 in Kaposi sarcoma-associated herpesvirus-linked disorders. Blood 2001;97(7):2173-6.
• Yarchoan R, Little RF. Immunosuppression-related malignancies. In: DeVita Jr. VT, Hellman S, Rosenberg SA, eds. Cancer, Principles and Practice of Oncology 6th Edition. Philadelphia: Lippincott Williams and Wilkins; 2001:2575-97.

Eponymous diseases

Castleman Tumor