多中心性巨大淋巴结增生症六例报道并文献复习
Case report of six multicentric Castleman's disease and related literature review
摘要目的 回顾性分析6例多中心性巨大淋巴结增生症(MCD)患者的诊断与治疗方法,并就相关文献进行分析.方法 6例初次诊断的MCD患者,采用干扰素和(或)联合化疗方案治疗.详细记录并分析其临床表现、实验室检查结果及治疗反应.结果 6例患者均为HIV阴性,其中4例病理学分型为浆细胞型,2例为混合型;6例均表现为多部位浅表淋巴结肿大、多克隆免疫球蛋白增多、低白蛋白血症.干扰素治疗2例,其中1例完全缓解,另1例激素及联合化疗效果均不满意,采用干扰素治疗3个月达到持续的部分缓解;其他4例采用联合化疗,其中1例死于疾病进展,3例部分缓解.结论 干扰素及联合化疗是最方便有效的治疗MCD的方法;血清白蛋白水平降低是MCD的诊断和监测指标.
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abstractsObjective To retrospectively analyze the diagnosis and treatment of multicentric Castleman's disease (MCD) ,and review related literatures. Methods A total of six patients were first-ever diagnosed as MCD and treated with combination chemotherapy and/or interferon α. The clinical manifestation, laboratory findings and therapeutic strategies were recorded in detail. Results In the six HIV-negative patients, histologically, four of them were diagnosed with plasma cell type of Castleman's disease, two with mixed type. All the six patients showed multiple lymphadenopathy, polyclonal hypergammaglobulinemia and hypoalbuminemia. One of the two patients treated with interferon α achieved complete remission, and the other,who showed no effects with hormone and combination chemotherapy ,achieved sustained partial remission after treatment with interferon α for 3 months. Of the four patients treated with combination chemotherapy, three achieved partial remission, and one died of no effects. Conclusions Interferon α and combination chemotherapy might be the most effective and convenient therapeutic methods for MCD. Serum albumin level may be used as a diagnostic and monitoring index for MCD.
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