Multicentric Castleman Disease

Multicentric Castleman disease (MCD) is a rare disease that affects the lymph nodes and related tissues. It is a form of Castleman disease that is "systemic" and involves multiple regions of lymph nodes (as opposed to unicentric Castleman disease, which involves a single lymph node or single region of lymph nodes). The signs and symptoms of MCD are often nonspecific, and are mild in some people but life-threatening in others. Symptoms may include enlarged lymph nodes in multiple regions, fever, weight loss, nausea, rash, and/or an enlarged large liver and spleen. The disease is diagnosed based on the symptoms present, laboratory test results, imaging studies, and results of a biopsy of the lymph nodes which shows specific features when studied under the microscope. In some cases, MCD is caused by human herpesvirus-8 (HHV-8) and is referred to as HHV-8-associated MCD. In other cases, the cause is not known and it is referred to as HHV-8 negative MCD, or idiopathic MCD (iMCD). Treatment of MCD is challenging, and treatment options and outcomes depend on the type and severity in each person.