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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: The pathogenesis of multicentric Castleman’s disease (MCD) is unknown and the diagnostic criteria are not uniform. Combined with the diagnosis and treatment of 10 cases of MCD cases, the diagnosis and treatment of the disease criteria to be explored. Methods: All 10 patients with primary MCD were diagnosed. The pathological changes of lymph nodes were in accordance with the changes of CD. The clinical manifestations, laboratory findings and treatment response were recorded and analyzed in detail. Results: All 10 patients were negative for HIV. Six of them were plasmacytoma type and four were mixed type. All of them showed multiple superficial lymph nodes, polyclonal immunoglobulin, hypoalbuminemia . Interferon therapy in 6 cases, of which 2 were completely relieved, 4 patients partially relieved, of which 1 hormone and combined chemotherapy were not satisfied with the use of interferon for 3 months to achieve sustained partial response; the other 4 cases with combination chemotherapy, in which One patient died of disease progression and three patients were partially relieved. CONCLUSION: The pathological characteristics of CD plasma cells or mixed type, polyclonal hyperglycemia and serum albumin level are the diagnostic and monitoring indexes for the diagnosis of MCD.