Castleman disease is a rare lymphoproliferative disorder. It
encompasses a spectrum of clinical disorders that range from localised asymptomatic
lymph node enlargement (Unicentric) to widespread lymphadenopathy (multicentric)
associated with severe symptoms due to cytokine excess.
It is a potentially fatal
disease particularly if diagnosed late or treated sub-optimally. Its rarity is such that
many haematologists have limited experience in diagnosing and treating the disease. The
true incidence in the UK is unknown although estimated at 500 – 800 new cases/year. When
treatment has been required this has been variable without national guidance to support
it.