疑难病研究——幼儿Castleman’s病

Childhood Castleman's Disease

摘要:

Castleman's病是一种局限性或系统性血管淋巴滤泡增生性淋巴结病，儿童罕见，其病因和发病机制不明。临床表现为淋巴系病变，伴或不伴有全身症状。临床分为单中心型(UCD)和多中心型(MCD)。病理改变包括透明血管型、浆细胞型和混合型。本病确诊需依据淋巴结病理活检。MCD目前无有效治疗，激素和α-干扰素可能对部分病人有效。UCD进行手术切除或加放疗可获完全缓解。

Abstract:

Castleman's disease (CD) is a localized or systemic angiofollicular lymphoproliferative disorder of unknown etiology and pathogenesis and rarely seen in children. The disorder manifest lymph node hyperplasia with or without systemic symptoms and can be classified to unicentric CD (UCD) and muticentric CD (MCD). The histological features include three types: hyaline vascular, plasma cell and mixed variant. The diagnosis of CD was comfirmed by lymph node biopsy. No effective treatment for patients with MCD has been found so far and some of which may be respondent to corticosteroid and interferon α. The patients with UCD can be improved by surgical intervention with/or radiotherapy.