Miyazaki Daigo, Yazaki Masahide, Gono Takahisa, Kametani Fuyuki, Tsuchiya Ayako, Matsuda Masayuki, Takenaka Yoshiaki, Hosh Yoshinobu, Ikeda Shu-ichi
Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan.
Amyloid. 2008 Jun;15(2):125-8. doi: 10.1080/13506120802006229.
We report a 67-year-old male patient who suffered from nephrotic syndrome and progressive renal dysfunction with monoclonal gammopathy (IgMkappa). Renal biopsy demonstrated amyloid deposition in glomeruli. Immunohistochemical studies of the renal amyloid using a number of antibodies, including anti-lambda and anti-kappa light chains, AA, beta(2)-microglobulin, and transthyretin, showed negative findings. Biochemical analysis of the deposited amyloid fibrils in gastroduodenal mucosa revealed that the amyloid fibrils were composed of an immunoglobulin heavy chain variable region (VH) fragment belonging to the VH1 subgroup, and a diagnosis of AH amyloidosis was made. In our institute, three patients with AH amyloidosis including the present one have been identified during the past 2 years, so AH amyloidosis seems to be by no means a rare disorder.
我们报告了一名67岁男性患者，他患有肾病综合征以及伴有单克隆丙种球蛋白病（IgMκ）的进行性肾功能不全。肾活检显示肾小球内有淀粉样沉积。使用多种抗体，包括抗λ和抗κ轻链、AA、β2-微球蛋白和转甲状腺素蛋白，对肾淀粉样蛋白进行免疫组织化学研究，结果均为阴性。对胃十二指肠黏膜中沉积的淀粉样纤维进行生化分析发现，淀粉样纤维由属于VH1亚组的免疫球蛋白重链可变区（VH）片段组成，从而做出了AH淀粉样变性的诊断。在我们研究所，过去两年间已确诊了包括本例在内的3例AH淀粉样变性患者，因此AH淀粉样变性似乎绝不是一种罕见疾病。
