与非结晶性IgG-κ副蛋白血症相关的肾小球组织细胞增多症病例报告。
Case report of glomerular histiocytosis associated with non-crystalline IgG-kappa paraproteinemia.
作者信息
Kishore Aditya, McRae Susanna A, Telio David, Beaulieu Monica C
机构信息
Division of Nephrology, University of British Columbia, Vancouver, Canada.
Faculty of Healthy Sciences and Medicine, Bond University, Gold Coast, Australia.
出版信息
BMC Nephrol. 2025 Feb 5;26(1):56. doi: 10.1186/s12882-025-03986-8.
BACKGROUND
Monoclonal gammopathy of renal significance (MGRS) represents a range of disease processes arising from monoclonal proteins depositing in the kidney. These deposits vary and can be broadly grouped as containing a substructure or being non-organised. Their clinical phenotype can include proteinuria, haematuria, kidney injury and tubulopathies resulting in electrolyte changes.
CASE PRESENTATION
Crystal storing histiocytosis (CSH) is a rare form of MGRS that typically deposits in the interstitium but rarely in the glomerulus to cause progressive kidney disease. We report a case of a male with known monoclonal protein and progressive proteinuria, whose biopsy showed glomerular histiocytosis with non-crystallizing IgG kappa inclusions.
CONCLUSION
This case reviews an unusual case of a glomerular histiocytosis with non-crystallizing IgG kappa inclusions.
背景
具有肾意义的单克隆丙种球蛋白病（MGRS）代表了一系列由单克隆蛋白沉积于肾脏引起的疾病过程。这些沉积物各不相同，大致可分为含有亚结构或无组织的两类。其临床表型可包括蛋白尿、血尿、肾损伤以及导致电解质改变的肾小管病。
病例介绍
晶体贮积性组织细胞增多症（CSH）是MGRS的一种罕见形式，通常沉积于间质，但很少沉积于肾小球，从而导致进行性肾病。我们报告一例已知患有单克隆蛋白和进行性蛋白尿的男性病例，其活检显示肾小球组织细胞增多症伴非结晶性IgG κ包涵体。
结论
本病例回顾了一例伴有非结晶性IgG κ包涵体的肾小球组织细胞增多症的罕见病例。
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