What are different types of Castleman disease?
What are different types of Castleman disease?
- Unicentric Castleman disease (UCD): This form affects a single or multiple lymph nodes in one region of the body. It is also called localized Castleman disease. The cause of UCS remains unclear.
- Multicentric Castleman disease (MCD): In this form, multiple lymph node regions in the body are involved. Approximately half of MCD cases are caused by HHV-8 infection in people with human immunodeficiency virus (HIV) or otherwise immunocompromised for other reasons. The remaining half of MCD cases are HHV-8 negative and are referred to as HHV-8 negative MCD or idiopathic MCD or iMCD. iMCD can be further classified intro three distinct clinical groups:
- iMCD associated with POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder and Skin changes)
- iMCD associated with TAFRO (Thrombocytopenia, Anasarca, Myelofibrosis, Renal dysfunction and Organomegaly)
- iMCD, not otherwise specified (iMCD-NOS)