Castleman's disease

Keywords Name of the disease and synonyms Names of excluded diseases Diagnostic criteria/Definition Comments on the differential diagnosis Incidence Clinical description Management/Treatments Etiology Biological diagnostic methods Unresolved questions and comments References Abstract Castleman's disease or angiofollicular lymphoid hyperplasia is a rare disease with two identified forms. The localized form, often pauci-symptomatic, is characterized by an isolated enlarged lymph node that regresses without sequelae after surgical excision. The multicentric form frequently presents general signs, polyadenopathy, organomegaly and sometimes a POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin) syndrome. The prognosis of the latter form is much less favorable and treatment often requires chemotherapy. The etiology of Castleman's disease remains unknown but recent reports have indicated a role of human herpesvirus 8 (HHV-8), especially in certain multicentric forms.