Shahidi H, Myers J L, Kvale P A
Department of Internal Medicine, Henry Ford Hospital, Detroit, Michigan, USA.
Mayo Clin Proc. 1995 Oct;70(10):969-77. doi: 10.4065/70.10.969.
To describe the two variants of Castleman's disease--the hyaline-vascular type and the plasma-cell type--and discuss the associated histologic features.
We present a case of the hyaline-vascular type and review the literature.
Castleman's disease was once thought to be localized and self-limited, but in recent years, reports have described a multicentric variety with severe systemic manifestations and, at times, an inexorable clinical course. Unlike the localized type for which surgical excision is curative regardless of the histologic type, multicentric disease often necessitates aggressive systemic therapy and portends a poor outcome. Little is known about the cause of this disorder, but the bulk of evidence points toward faulty immunoregulation that results in excessive proliferation of B lymphocytes and plasma cells in lymphoid organs.
Castleman's disease is rare and poorly understood. The diagnosis is "contextual" and must be considered in the appropriate clinical setting and only after all other causes of lymphadenopathy have been investigated and excluded. The optimal therapeutic regimen is unknown.
描述卡斯特曼病的两种变体——透明血管型和浆细胞型，并讨论相关的组织学特征。
我们报告一例透明血管型病例并复习文献。
卡斯特曼病曾被认为是局限性且自限性的，但近年来，有报道描述了一种多中心型，伴有严重的全身表现，有时临床病程不可阻挡。与局限性类型不同，无论组织学类型如何，手术切除对局限性类型具有治愈性，而多中心型疾病通常需要积极的全身治疗，且预后不良。关于这种疾病的病因知之甚少，但大量证据表明是免疫调节功能异常导致淋巴器官中B淋巴细胞和浆细胞过度增殖。
卡斯特曼病罕见且了解甚少。诊断是“基于背景的”，必须在适当的临床背景下考虑，并且只有在对所有其他淋巴结病病因进行调查并排除后才能做出诊断。最佳治疗方案尚不清楚。
