A Case of Plasma Cell Type Giant Lymph Node Hyperplasia (Castleman's Disease) / 대한소아혈액종양학회지

ABSTRACT

Castleman's disease was first described in 1956 in a group of patients with localized mediastinal lymph node enlargement characterized by hyperplasia of lymphoid follicles and marked capillary proliferation with endothelial hyperplasia. They have been divided into 2 histologic types the hyaline-vascular type, which was more common and usually asymptomatic, was characterized by small hyaline-vascular follicles and interfollicular capillary proliferation; the plasma-cell type was characterized by large follicles with intervening sheets of plasma cells. Systemic manifestations, such as fever, anemia and hyperglobulinemia, were frequently associated with the plasma cell type. Localized lesions have behaved in a benign fashion, and complete surgical excision has been curative. But recent years, reports have described a multicentric variety with severe systemic manifestations, exorable clinical course and poor outcome. Although Castleman's disease has been described at all ages, the disease is rare in childhood. This paper describes a case of plasma cell type Castleman's disease in a 12-year-old boy and review of the literature. We conclude that the Castleman's disease must be considered in childhood lymphadenopathy and the clinicians should be mindful of the malignant potential of the disease and their possible multicentricity. Appropriate treatment plan, close follow-up and periodic surveillance are necessary.