Abstract
We report Castleman's disease in a 48-year-old male who complained of rapidly enlarging mass on the right side on the neck. He presented unusual association of thrombocytopenia and the facio-scapulo-humeral type of progressive muscular dystrophy. The mass was extirpated, and histopathological diagnosis was the hyaline-vascular type of Castleman's disease. Following resection, thrombocytopenia was ameliorated, but progressive muscular dystrophy did not improve. We suggest that the former was the result of the platelet-trapping mechanism often seen in hemangioma, and that the latter was coincidental.
Immunological study with a panel of monoclonal antibodies and polyclonal antisera was performed to clarify the nature of this heterogeneous condition. This mass was found to be a polyclonal proliferation composed chiefly of B-cells bearing IgD, IgM, and IgA on their membranes. Helper T cells outnumbered suppressor T cells at the ratio of 2: 1. With reference to the recent literature, we suggest that the hyaline-vascular type of Castleman's disease is an atypical hyperplasia possibly resulting from abnormal immunologic response to antigenic stimulation.
