Castleman Disease

Castleman disease is a rare group of lymphoproliferative disorders characterised by abnormal enlargement of lymph nodes due to excessive growth of immune cells. Although it is not considered a typical cancer, the disease can behave aggressively and significantly affect the immune system. Castleman disease is classified into two main forms: unicentric Castleman disease (UCD), involving a single group of lymph nodes, and multicentric Castleman disease (MCD), affecting multiple lymph node regions and causing widespread inflammation.

The exact cause varies depending on the type. Multicentric Castleman disease may be associated with infection by human herpesvirus-8 (HHV-8), particularly in people with weakened immune systems, while some cases are idiopathic with no identifiable cause. Common symptoms include painless swollen lymph nodes, persistent fever, night sweats, fatigue, unexplained weight loss, enlarged liver or spleen, and anaemia. Patients with UCD may have few or no symptoms until the enlarged lymph node is discovered during imaging or examination.

Diagnosis involves blood tests, imaging studies such as CT or PET scans, and lymph node biopsy to confirm the characteristic tissue changes. Treatment depends on the disease type and may include surgical removal of affected lymph nodes, corticosteroids, immunotherapy, targeted monoclonal antibodies, antiviral therapy when appropriate, or chemotherapy for severe multicentric disease. Early diagnosis and personalised treatment improve symptom control and long-term outcomes.