中华临床免疫和变态反应杂志2017，Vol.11Issue(2)：126-130,5.DOI:10.3969/j.issn.1673-8705.2017.02.005
原发性胆汁性胆管炎并发浆细胞病患者临床特点
Characteristics of Patients with Primary Biliary Cholangitis Complicatedwith Plasma Cell Dyscrasia
摘要
Abstract
Objective To investigate the clinical features of patients diagnosed of primary biliary cholangitis (PBC) complicated with plasma cell dyscrasia.Methods Patients diagnosed of PBC complicated 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 patients 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 electrophoresis identified IgG λ in 2 patients, IgG κ in 1 patient, IgA λ in 2 patients, and IgM κ in 1 patient.Conclusions PBC patients could present with plasma cell dyscrasia, 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.关键词
原发性胆汁性胆管炎/原发性胆汁性肝硬化/浆细胞病/多发性骨髓瘤Key words
primary biliary cholangitis/primary biliary cirrhosis/plasma cell dyscrasia/multiple myeloma分类
医药卫生引用本文复制引用
孙媛媛,边赛男,张奉春..原发性胆汁性胆管炎并发浆细胞病患者临床特点[J].中华临床免疫和变态反应杂志,2017,11(2):126-130,5.基金项目
国家自然科学基金(81571594),国家重点研发计划重点专项(2016YFA0101003) （81571594）
