多中心性Castleman病的诊断标准探讨

Discussion of diagnosis criteria of multicentric Castleman's disease

目的:多中心性巨大淋巴结增生症(multicentric Castleman's disease,MCD)发病机制不明,诊断标准不统一。结合我院诊治的10例MCD病例,就本病的诊治标准进行探讨。方法:全部10例均为初次诊断的MCD患者,淋巴结病理符合CD改变,详细记录并分析患者的临床表现、实验室检查结果及治疗反应。结果:10例患者均为HIV阴性,其中6例病理学分型为浆细胞型,4例为混合型;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6例,其中2例完全缓解,4例部分缓解,其中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:CD浆细胞型或混合型病理学特征、多克隆高免疫球蛋白血症及血清白蛋白水平降低是诊断MCD的诊断和监测指标。

Objective：Until now,there are no uniform diagnosis criteria for multicentric Castleman＇s disease（MCD）,a rare lymphoproliferative disorder with aggressive results.The purpose of current study was to report our experiences of the treatment in 10 patients diagnosed as MCD in our hospital,and to discuss the criteria of diagnosis.Method：A total of ten patients were diagnosed according to the criteria of literatures,and treated with combination chemotherapy and/or interferon α.The clinical manifestation,laboratory findings and therapeutic strategies were recorded in detail.Result：In these ten HIV negative patients,six of them were histologically diagnosed as plasma cell type,while four as mixed type.All the patients showed multiple lymphadenopathy,polyclonal hypergammaglobulinemia and hypoalbuminemia,and met the diagnosis criteria of MCD.Two of six patients treated with interferon α achieved complete remission,and two patients achieved sustained partial remission.Of four patients treated with combination chemotherapy,three achieved complete remission,one achieved partial remission,and one died of no effects.The serum albumin recovered to normal level in all patients achieving partial remission and complete remission.Conclusion：Multiple lymphadenopathy,polyclonal hypergammaglobulinemia,hypoalbuminemia and historically plasma cell type or mixed type of Castleman′s disease are necessary diagnostic indexes.Serum albumin level may be employed as monitoring index for illness status in this disorder.