Castleman's Disease in Children: The Experience of a Children's Hospital in Africa

Abstract

As the incidence of childhood Castleman's disease (CD) is reported to be rare in Western communities, the recent occurrence of three cases in non-HIV infected prepubertal children stimulated a 10-year (1983–1993) retrospective investigation into the conditions at a children's hospital that serves a predominantly developing community. Retrospective histologic examination revealed six cases of CD among children 3–11 years of age and another five probable cases having histological features of CD without adequate clinical and laboratory data. Three CD cases were associated with malignancy (Kaposi's sarcoma in two and a gastric B cell lymphoma in the other). Two CD cases were plasma cell type, one hyaline vascular, and three intermediate. One child with recurrent plasma cell-type features in a groin node had a Kaposi's sarcoma in the thigh. The five probable cases of CD all had intermediate histological features. Four of the 11 cases being reported came from a geographic locality that provides about 3% of the laboratory's case material and these included both cases that developed Kaposi's sarcoma. All patients who developed malignancy had the plasma cell variant. As all cases have been identified in developing communities, where the disease may often be misdiagnosed as nonspecific reactive lymphadenopathy, and there is an evident association with malignancy, it is recommended that children in these areas with the plasma cell variant of CD be closely monitored for subsequent malignancy.

Key Words:

• angiofollicular lymphoid hyperplasia
• Castleman's disease
• developing countries
• infancy and childhood
• Kaposi's sarcoma
• lymphoma—B cell