Fuchs A, Jagirdar J, Schwartz I S
Am J Clin Pathol. 1987 Sep;88(3):302-7. doi: 10.1093/ajcp/88.3.302.
Amyloidosis has been increasingly recognized in association with renal failure and chronic hemodialysis. This report describes three patients who had long-term hemodialysis (between 7-18 years), in whom deposits developed of a new type of amyloid of beta 2-microglobulin origin. Beta 2-microglobulin amyloid (AB2M) was found in multiple organs, i.e., bone, subendocardium, gastrointestinal blood vessels, tongue, and carpal tunnel connective tissue. AB2M displayed characteristic amyloid features on conventional light and polarized microscopic examination after congo red staining. However immunostaining with anti-amyloid A protein, kappa, and lambda antisera were negative. The studied material reacted positively with beta 2-microglobulin antisera, identifying AB2M in all three cases. Ultrastructural study revealed an unusual curvi-linear fibrillar configuration. AB2M appears to be a new subtype of systemic amyloidosis secondary to renal failure and long-term hemodialysis.
淀粉样变性与肾衰竭和慢性血液透析的相关性已越来越受到认可。本报告描述了3例长期血液透析（7至18年）的患者，他们体内出现了一种源自β2-微球蛋白的新型淀粉样沉积物。在多个器官中发现了β2-微球蛋白淀粉样蛋白（AB2M），即骨骼、心内膜下、胃肠道血管、舌部和腕管结缔组织。刚果红染色后，AB2M在传统光镜和偏振显微镜检查中呈现出典型的淀粉样特征。然而，用抗淀粉样蛋白A、κ和λ抗血清进行免疫染色均为阴性。所研究的材料与β2-微球蛋白抗血清反应呈阳性，在所有3例中均鉴定出AB2M。超微结构研究显示出一种不寻常的曲线状纤维结构。AB2M似乎是肾衰竭和长期血液透析继发的系统性淀粉样变性的一种新亚型。
