Multicentric Castleman’s disease: a rare case presentation

Abstract

Castleman’s disease (CD), also known as Angiofollicular lymph node hyperplasia, is a rare lymphoproliferative disorder first reported by dr. Benjamin Castleman in 1956. The estimated incidence rate is 5 to 25 per million person-years. Histologically, the CD can be classified as hyaline-vascular type, plasma cell type and mixed type. HHV-8-associated MCD (Multicentric CD) is most commonly diagnosed in HIV-infected or otherwise immunocompromised individuals. Co-occurrence of polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder and skin changes is known as POEMS syndrome. Treatment of iMCD (idiopathic CD) is challenging, and outcomes are poor because of no uniform treatment guidelines. The anti-interleukin-6 monoclonal antibody Siltuximab with or without corticosteroids is the preferred first-line therapy for iMCD.