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单克隆丙种球蛋白病中伴有肝脏和骨髓大量纤维样沉积的免疫触须样肾小球病。

Immunotactoid-like glomerulopathy with massive fibrillary deposits in liver and bone marrow in monoclonal gammopathy.

作者信息

Strøm E H, Hurwitz N, Mayr A C, Krause P H, Mihatsch M J

机构信息

Institute for Pathology, University of Basel, Switzerland.

出版信息

Am J Nephrol. 1996;16(6):523-8. doi: 10.1159/000169053.

DOI:10.1159/000169053
PMID:8955765
Abstract

At autopsy, massive nonamyloid fibrillar deposits, immunoreactive to IgG and kappa light chain, were found in glomeruli, liver, and bone marrow of a 72-year-old woman. The patient suffered from severe nephrotic syndrome, hepatomegaly and cholestasis, normochromic anemia, and IgG kappa monoclonal gammopathy. Fibrillary glomerulopathies, most often denoted as fibrillary glomerulonephritis or immunotactoid glomerulopathy, are generally considered to have deposits restricted to the glomeruli. However, this study indicates that fibrillary deposits may be a systemic manifestation of fibrillary glomerulonephritis or immunotactoid glomerulopathy, at least when the patient is suffering from a monoclonal gammopathy.

摘要

尸检时,在一名72岁女性的肾小球、肝脏和骨髓中发现了大量非淀粉样纤维沉积物,对IgG和κ轻链具有免疫反应性。该患者患有严重的肾病综合征、肝肿大和胆汁淤积、正色素性贫血以及IgG κ单克隆丙种球蛋白病。纤维性肾小球病,通常被称为纤维性肾小球肾炎或免疫触须样肾小球病,一般认为沉积物仅限于肾小球。然而,这项研究表明,纤维沉积物可能是纤维性肾小球肾炎或免疫触须样肾小球病的一种全身性表现,至少在患者患有单克隆丙种球蛋白病时是这样。

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引用本文的文献

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Molecular study of an IgG1kappa cryoglobulin yielding organized microtubular deposits and glomerulonephritis in the course of chronic lymphocytic leukaemia.慢性淋巴细胞白血病过程中产生有组织微管沉积物和肾小球肾炎的IgG1κ冷球蛋白的分子研究
Clin Exp Immunol. 2002 Jul;129(1):113-8. doi: 10.1046/j.1365-2249.2002.01896.x.