Two Cases of Castlema's Disease in Childern

소아에 발생한 Castleman's Disease 2례

Castleman's disease is an atypical lymphoproliferative disorder of unknown origin. It has three histologic variants(hyaline vascular, plasma-cell, and mixed) and two clinical types(localized and multicentric). Some sufferers have constitutional symptoms and laboratory abnormalities such as anemia, hypoalbuminemia, hypergammaglobulinemia, and elevated erythrocyte sedimentation rate. The localized form is cured by complete surgical excision whereas the multicentric form is managed by prednisone and other immunosuppressor drugs. The prognosis of the multicentric form is worse than the localized form since malignancies and severe infections may lead to a rapidly fatal outcome. Castleman's disease has been rarely reported at pediatric age in Korea. We experienced two cases of Castleman's disease detected at 3 and 5 years of age. They were presented with painless enlargement of submandibulars and axillary lymph nodes but had no associated symptoms. The lesions were excised and diagnosed as Castleman's disease, and no recurrence was noted during follow-up periods.

저자는 우연히 발견된 무통성 종괴로 내원한 환아에서 소아과 영역에서 드문 Castleman 씨 병 2례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다. 림프절 종창으로 추정되는 무통성의 종괴를 보이는 환아에서는 Castleman 씨 병을 감별 진단으로 반드시 고려해 보아야 할 것이다.