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作 者:程虹[1] CHENG Hong.(Division of Nephrology, Beijing Anzhen Hospital, CapitaI Medical University, Beijing 100029, China)
机构地区:[1]首都医科大学附属北京安贞医院肾内科,北京100029
出 处:《中国实用内科杂志》2018年第6期515-518,共4页Chinese Journal of Practical Internal Medicine
摘 要:Ⅰ型及Ⅱ型冷球蛋白血症(CG)发病均与单克隆免疫球蛋白(MIg)相关。Ⅰ型冷球蛋白由单一的MIg组成,Ⅰ型CG的基础疾病为B细胞淋巴增殖性疾病,多数为意义未明的丙种球蛋白病(MGUS),其次为多发性骨髓瘤及华氏巨球蛋白血症等恶性病;Ⅱ型冷球蛋白由具有类风湿因子活性的单克隆IgM与多克隆IgG组成,Ⅱ型CG的基础疾病绝大多数为感染性疾病(丙型肝炎病毒感染最常见),少数为干燥综合征等自身免疫性疾病。它们继发的肾炎(GN)主要为膜增生性肾小球肾炎。文章对这两型CG及其GN的发病机制、临床及病理表现和治疗原则做一讨论。The pathogenesis of type I and type II cryoglobulinemia(CG) is related to monoclonal immunoglobulin(MIg). Type I cryoglobulin is composed of single MIg and type I CG is caused by B cell linphoproliferative diseases, of which the principal disease is monoclonal gammopathy of unknown significance(MGRS) and the next is malignant diseases such as multiple myeloma and Waldenstr?m macroglobulinemia. Type II cryoglobulin consists of monoclonal Ig M with rheumatoid factor activity and polyclonal Ig G. The vast majority of type I CG are caused by the chronic infection(especially hepatitis C virus infection), and a small part are caused by autoimmune diseases, for example, Sj?gren's syndrome. The main pathological pattern of CG-associated glomerulonephritis(CG-GN) is membranoproliferative glomerulonephritis. The pathogenesis, clinical and pathological manifestations, and treatment of these two types of CG and CG-GN will be discussed in this paper.
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