12例多中心型Castleman 病的诊断与治疗

目的:探讨多中心型Castleman病的临床表现、诊断及治疗方法。方法:对2001年10月~2009年5 月中国医科大学附属第一医院收治的12例多中心型Castleman病例的诊疗过程及预后进行了回顾性分析。结果:多中心型Castleman病累及多个器官或系统，临床表现复杂多样，亦无特异性影像学表现或实验室指标，易漏诊、误诊。组织病理学上，Castleman病分为透明血管型、浆细胞型及混合型三种亚型。12例病例均经活检病理确立诊断和组织分型，三种组织病理分型分别占58% 、33% 及8% 。12例多中心型Castleman病，有随访结果的10例，完全缓解2 例（透明血管型）；带病生存5 例（透明血管型3 例，浆细胞型2 例）；死亡3例（浆细胞型2 例，混合型1 例）。 中位生存时间为17个月。结论:多中心型Castleman病早期诊断是关键，确诊主要依靠病理。活检时注意选择不同部位、表现典型的淋巴结手术切取，必要时多次送检。治疗上以系统治疗为主，疗效不确切，个体差异大，预后不佳。尚在探究中的新型制剂也许会为多中心型Castleman病的治疗带来希望。 

Diagnosis and Treatment of Multicentric Castleman's Disease-Study of 12 cases

Objective: To investigate the clinical manifestations, diagnosis and treatment of multicentric Castleman's disease. Methods:A retrospective review of 12patients admitted to the First Hospital of China Medical University during the period from October 2001 through May 2009 with the histopathologic diagnosis of Castleman's disease was performed. The clinical course of diagnosis and treatment and the outcome of this uncommon clinicopathologic entity were analyzed. Results: Multicentric Castleman's disease is characterized by polylymphadenopathy and involvement of multiple organs and systems, and it is far more varied in its clinical course than localized disease. The 12-case study group is classified into 3 histopathologic groups: hyaline-vascular, plasma cell, and mixed variant, accounting for 58%,33%, and8% of the patients, respectively. Among the 10multicentric cases with follow-up data available,2 cases achieved complete remission (with the pathologic subtype of hyaline-vascular variant), 5 were alive with disease still present (3 with hyaline-vascular variant and 2 with plasma cell variant), while3 other cases died (2 with plasma cell variant and one with mixed variant). Conclusion : Accurate diagnosis of multicentric Castleman's disease is crucial and is preferably done by histopathological evaluation of an excised specimen. On the other hand, to make a diagnosis at an earlier stage, various sites on typical lymph nodes can be selected and if necessary be repeatedly biopsied. Unlike surgical resection which can cure the localized type, the multicentric type, with poorer prognosis, is treated mainly with systemic therapy. Although new treatment strategies are being explored based on underlying pathophysiology, optimal standard therapies have not yet been established for multicentric Castleman's disease.