摘要
目的探讨原发性胆汁性胆管炎(primary biliary cholangitis,PBC)并发浆细胞病患者的临床特点。方法回顾性分析2002年1月1日至2016年7月31日于北京协和医院诊断PBC并发浆细胞病的住院患者,分析其临床特征、实验室检查、治疗及结局,并进行文献复习。结果 PBC并发浆细胞病共6例患者,女性5例,男性1例,平均年龄(62±10)岁。1例患者为诊断PBC的同时诊断浆细胞病,5例患者为PBC之后诊断浆细胞病,中位时间14个月(范围12~48个月)。3例患者免疫球蛋白(immunoglobulin,Ig)M升高,2例患者IgA升高,1例患者Ig G升高。5例患者抗线粒体抗体阳性,2例患者抗着丝点抗体阳性。3例患者为多发性骨髓瘤(multiple myeloma,MM),2例患者为意义未明的单克隆免疫球蛋白血症(monoclonal gammopathy of undetermined significance,MGUS),1例患者为巨球蛋白血症。血免疫固定电泳提示Ig Gλ2例,Ig Gκ1例,IgAλ2例,Ig Mκ1例。结论 PBC患者可出现浆细胞病,在初诊及病程中均可出现,以MM及MGUS多见以及Ig G及IgA型多见。临床医师应注意对该类患者免疫球蛋白水平的监测,警惕浆细胞病的发生。
Objective To investigate the clinical features of patients diagnosed of primary biliary cholangitis （PBC） complicated with plasma cell dyscrasia. Methods Patients diagnosed of PBC compli- cated with plasma cell dyscrasia in Peking Union Medical College Hospital between January 1st, 2002 and July 31st, 2016 were retrospectively included. Clinical manifestations, laboratory examinations, treatment, and outcomes of these patients were analyzed. A literature review was also done. Results Among the 6 pa- tients diagnosed of PBC and plasma cell dyscrasia, 5 were female and the mean age was （62±10） years. One patient was diagnosed of PBC and plasma cell dyscrasia simultaneously, and the remaining 5 patients were diagnosed of plasma cell dyscrasia after the diagnosis of PBC with a median interval of 14 months （range 12-48 months）. Three patients had increased immunoglobulin M （IgM）, 2 patients had increased IgA, and 1 patient had increased IgM. Five patients presented with anti-mitochondrial antibody （AMA） and 2 patients presented with anti-centromere antibody （ACA）. Multiple myeloma （MM） was diagnosed in 3 patients, monoclonal gammopathy of undetermined significance （MGUS） was diagnosed in 2 patients, and Waldenstrom macroglobulinemia was diagnosed in 1 patient. Serum immunofixation electro- phoresis identified IgG h in 2 patients, IgG K in 1 patient, IgA h in 2 patients, and IgM K in 1 patient. Conclusions PBC patients could present with plasma cell dyserasia, either at of the time of diagnosis or during the disease course. MM and MGUS are the common type, with IgG and IgA sub-types. Clinicians should monitor immunoglobulins in PBC patients and pay attention to plasma cell dyscrasia.
出处
《中华临床免疫和变态反应杂志》
2017年第2期126-130,共5页
Chinese Journal of Allergy & Clinical Immunology
基金
国家自然科学基金(81571594)
国家重点研发计划重点专项(2016YFA0101003)
