Castleman’s Disease

Castleman’s disease is a rare lymphoproliferative disorder characterised by angiofollicular lymphoid hyperplasia. Two histological variants are recognised; a hyaline vascular variant and a less common plasma cell variant. Castleman’s disease has germinal centre hyalinisation or atrophy surrounded by concentric layers of lymphocytes with prominent vascular hyperplasia, hyalinisation of small vessels and interfollicular sheets of plasma cells and immunoblasts.

Multicentric Castleman’s disease (MCD) presents with constitutional symptoms including fever, weight loss and night sweats and clinical findings include lymphadenopathy, hepatosplenomegaly and rashes. Investigations frequently reveal microcytic anaemia, hypoalbuminaemia and polyclonal hypergammaglobulinaemia.

Many of the paraneoplastic manifestations of Castleman’s disease are believed to be due to excess interleukin-6 (IL-6) production by the tumour, possibly from the viral IL-6 homologue gene of HHV8.

The best treatment for MCD is not known.