Kasahara N, Tamura H, Matsumura O, Nagasawa R, Suzuki Y, Ohgida T, Itoyama S, Mitarai T, Isoda K
Fourth Department of Internal Medicine, Saitama Medical School, Kawagoe.
Intern Med. 1994 Apr;33(4):216-21. doi: 10.2169/internalmedicine.33.216.
This report describes a case of light chain deposition disease (LCDD) with unusual findings of fibrillar structures in the deposits and marked calcification in several organs. A forty-year-old man was initially diagnosed with LCDD in 1987, and died of sepsis three and one-half-years later. Histological examination of autopsy specimens demonstrated eosinophilic amorphous materials, which differed from amyloid, in vessel walls or around parenchymal cells in almost every organ examined. Ultrastructurally, in addition to granular deposits, fibrillar structures were also seen in the deposits. Marked calcification was present in the myocardium, skeletal muscles, adrenal glands and arteries.
本报告描述了一例轻链沉积病（LCDD），其沉积物中有纤维状结构这一不寻常发现，且多个器官出现明显钙化。一名40岁男性于1987年最初被诊断为LCDD，三年半后死于败血症。尸检标本的组织学检查显示，在几乎每个检查的器官的血管壁或实质细胞周围有嗜酸性无定形物质，这些物质与淀粉样蛋白不同。超微结构上，除颗粒状沉积物外，沉积物中还可见纤维状结构。心肌、骨骼肌、肾上腺和动脉中存在明显钙化。
