[Angiofollicular lymph node hyperplasia (Castleman disease)].

Angiofollicular lymph node hyperplasia (Castleman's disease) is a relatively rare disease of differential diagnostic interest in patients with lymphadenopathy. The etiology and pathogenesis are still not elucidated. The disease is classified into localized and systemic types. The localized form is divided histologically into hyaline-vascular type and plasma cell types. The former is usually demonstrated incidentally as a widening of the mediastinum in otherwise asymptomatic patients. The plasma cell type usually presents in the abdominal lymph nodes and is accompanied by fever, loss of weight, anemia and hypergammaglobulinemia. Surgical treatment is curative in the localized disease. The systemic disease involves multiple lymph nodes, and multiple organs are affected. The prognosis is dubious, and frequently the patients rapidly die from septicemia or other infectious complications. Some patients develop malignancies. Treatment with glucocorticosteroids and chemotherapy has only demonstrated a limited effect. The diagnosis requires both a characteristic histopathology and typical clinical symptoms. A current illustrative case report is presented.