Die angiofollikuläre Lymphadenopathie Castleman

Abstract

The Castleman Disease, also known as angiofollicular lymph node hyperplasia is a rare disorder that results in abnormal proliferation of B-lymphocytes and plasma cells in lymphoid organs. It was first described as a pathologic entity in 1954 and later defined by Castleman et al. in 1956. The Castleman's disease is characterised by lymph node hyperplasia with vascular proliferation and endothelial hyperplasia. On the basis of histologic criteria it has been divided into hyaline-vascular and plasma cell variant. The hyaline-vascular type (90 % of cases), generally benign, occurs in younger patients with a localised mediastinal mass - surgical excision is curative. The plasma cell variant (10 % of cases), localised or multicentric, is generally aggressive, and occurs in older patients with generalised lymphadenopathy, hepatosplenomegaly, systemic symptoms, anaemia, elevated inflammatory indices, and a polyclonal rise in immunoglobulins. The prognosis is poor despite the Chemotherapy. Surgery is not indicated. We present two cases of unicentric Castleman Tumor of hyaline-vascular type. The patients were cured by surgical resection.