目的 通过病例资料分析IgG4相关疾病特点,总结IgG4相关疾病被误诊为多中心型Castleman病的原因,提高对此类疾病的诊断准确性.方法 回顾性分析2008年1月至2012年12月北京协和医院诊治的3例IgG4相关疾病患者误诊为多中心型Castleman病的临床资料.结果 3例患者均为男性,年龄为53～57岁,均有全身广泛的淋巴结肿大及多系统受累,均曾行1次以上淋巴结活检.经病理检查,2例确诊、1例疑诊为多中心型Castleman病.筛查3例患者血清IgG4水平均明显升高,均大于1350 mg/L;正电子发射计算机断层显像仪-CT(positron-emission tomography/computed tomography,PET-CT)显示全身广泛淋巴结及多脏器摄取增高;2例患者病理免疫组化证实IgG4阳性淋巴细胞占淋巴细胞50%以上.3例患者最终被确诊为IgG4相关疾病.经足量糖皮质激素及环磷酰胺治疗后3例患者的临床、血清学及影像学异常均迅速显著缓解.结论 IgG4相关疾病易被误诊为多中心型Castleman病,对临床表现、血清IgG4水平、PET-CT检查及病理免疫组化的综合分析有助于IgG4相关疾病的诊断.%Objective To analyze the clinical features of IgG4-related disease and determine the reasons why The patients with IgG4-related disease are misdiagnosed with multicentric Castleman's disease. Methods We retrospectively reviewed the medical records of the three patients with IgG4-related disease, who were admitted to Peking Union Medical College Hospital ( PUMCH) and had been misdiagnosed as multicentric Castleman' s disease. Results All three patients were males aged 53 ~ 57. They presented with generalized lymphadenopathy and multiple organ involvement. Biopsy results of their lymph nodes were consistent with multicentric Castleman' s disease. However, all their serum IgG4 levels were significantly elevated. Fluorodeoxyglucose (FDG) positron emission tomography-computed tomography (PET-CT) scan detected abnormal FDG uptake in multiple organs in all patients; Immunohistochemical analysis of biopsy specimen from two patients revealed that the IgG4-positive/IgG-positive plasma cell ratios were more than 50% . They were diagnosed with IgG4-related disease. High-dose corticosteroid with cyclophosphamide resulted in marked resolution of the physical, serological, and imaging abnormalities in all patients. Conclusions IgG4-related disease sometimes can be misdiagnosed as multicentric Castleman' s disease, if only based on clinical features and conventional pathological examination. It is necessary to strengthen the recognition of the disease. Measurement of serum IgG4 levels, PET-CT scan and specific immunohistochemical analysis for determining the IgG4-positive/IgG-positive plasma cell ratio are helpful in the diagnosis of IgG4-related disease.
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