Vorob'eva O A, Nast A, Cohen A
Arkh Patol. 2006 Nov-Dec;68(6):19-23.
The paper shows the morphological findings of and clinical and demographic data on 61 patients with nonamyloid form of renal monoclonal immunoglobulin deposition disease (MIDD) unassociated with amyloidosis and/or Bence-Jones nephropathy: 40 cases of light-chain deposition disease, 18 cases of light-and-heavy- chain deposition disease (LHCDD) and 3 cases of heavy-chain deposition disease (HCDD). According to the composition of paraprotein deposits, the cases were distributed as follows: k (30), lambda (10), IgG/k (6), IgA/k (6), IgG/lambda (4), IgA/lambda (2), and IgA/gamma (3). Light microscopy revealed three variants of the glomerular structure: diffuse nodular glomerulopathy (42.6%), diffuse mesangeal dilation (27.9%), and inract glomeruli (29.5%). Varying severity of tubular atrophy was noted in 95% of cases. Tubular, glomerular, and smooth muscle basement membrane deposits substantially differed in immunofluorescent (100%, 91.8% and 54.1%) and ultrastructural studies (55.7%, 45.9%, and 4.9%, respectively). Azotemia (68.9%) and proteinuria (55.8%) were most commonly revealed. The incidence of nephrotic syndrome concurrent with severe proteinuria was 27.9%.
本文展示了61例非淀粉样形式的肾单克隆免疫球蛋白沉积病(MIDD)患者的形态学发现以及临床和人口统计学数据,这些患者与淀粉样变性和/或本-周蛋白尿性肾病无关:40例轻链沉积病,18例轻链和重链沉积病(LHCDD)以及3例重链沉积病(HCDD)。根据副蛋白沉积物的组成,病例分布如下:κ(30例),λ(10例),IgG/κ(6例),IgA/κ(6例),IgG/λ(4例),IgA/λ(2例),以及IgA/γ(3例)。光镜检查显示肾小球结构有三种变体:弥漫性结节性肾小球病(42.6%)、弥漫性系膜扩张(27.9%)和正常肾小球(29.5%)。95%的病例观察到不同程度的肾小管萎缩。肾小管、肾小球和平滑肌基底膜沉积物在免疫荧光研究(分别为100%、91.8%和54.1%)和超微结构研究(分别为55.7%、45.9%和4.9%)中存在显著差异。最常见的表现是氮质血症(68.9%)和蛋白尿(55.8%)。并发严重蛋白尿的肾病综合征发生率为27.9%。