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[单克隆丙种球蛋白病的谱系]

[Spectrum of monoclonal gammopathies].

作者信息

Matuszkiewicz-Rowińska Joanna, Wieliczko Monika

机构信息

Klinika Nefrologii, Dializoterapii i Chorób Wewnętrznych, Warszawski Uniwersytet Medyczny, Warszawa, Polska.

出版信息

Wiad Lek. 2017;70(6 pt 2):1170-1174.

Abstract

The monoclonal gammopathies are defined as heterogenous group of diseases characterized by proliferation of a single clone of plasma cells, producing immunoglobulin or light (rarely heavy) chains, which can be detected in blood or urine as monoclonal (M) protein. The most common among them is monoclonal gammopathy of undetermined significance (MGUS), the asymptomatic benign disorder, present in ~ 3% of the population aged ≥50 years. However MGUS is a pre malignant condition and may progress to symptomatic multiple myeloma or related malignancies, with annual rate of approximately 1%. The clone may also produce kidney damage, resulting from just the protein M, with different patterns of renal disease. Since the lesions are progressive and may be severe leading to a significant morbidity the term "monoclonal gammopathy of renal significance (MGRS)" has been recently introduced.

摘要

单克隆丙种球蛋白病被定义为一组异质性疾病,其特征是单个浆细胞克隆增殖,产生免疫球蛋白或轻链(很少是重链),可在血液或尿液中检测到单克隆(M)蛋白。其中最常见的是意义未明的单克隆丙种球蛋白病(MGUS),这是一种无症状的良性疾病,在≥50岁的人群中约占3%。然而,MGUS是一种癌前状态,可能进展为有症状的多发性骨髓瘤或相关恶性肿瘤,年进展率约为1%。该克隆也可能导致肾脏损害,仅由M蛋白引起,有不同的肾脏疾病模式。由于病变是进行性的,可能很严重,导致显著的发病率,因此最近引入了“具有肾脏意义的单克隆丙种球蛋白病(MGRS)”这一术语。

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