Seventeen consecutive patients with dystrophic amyloidosis are reported here (eight Chinese, three Indian, three Iban, two Malay and one Caucasian). Ten were females and seven males, with ages ranging from 12 to 80 years (mean of 48 years). Five instances of dystrophic amyloidosis occurred in areas of tissue damage in the cardiovascular system, including fibrotic cardiac valves and an atheromatous plaque. Three occurred in osteoarthritic joint tissue. Of note were three occurrences in endometriotic cyst walls, four in the fibrotic walls of epidermal cysts, one in a hernial sac and one at the edge of a skin ulcer. All deposits were congophilic and exhibited green-birefringence and permanganate-resistance. Immunohistochemistry did not reveal reactivity for AA protein or immunoglobulin lambda or kappa light-chains. AP protein was detected in 35% of cases. Our results show that, besides the usual sites of osteoarthritic joints and damaged heart valves, dystrophic amyloidosis can complicate other areas of chronic tissue damage and fibrosis such as walls of cysts and ulcers. While the pathogenesis and biochemical nature remain unresolved, immunohistochemistry indicates that neither AA nor AL proteins are present in the deposits, and suggests that a different amyloid protein is involved.

译文

这里连续报道了17例营养不良性淀粉样变性患者 (八名中国人,三名印度人,三名伊班族,两名马来人和一名高加索人)。10名女性和7名男性,年龄在12至80岁之间 (平均48岁)。5例营养不良性淀粉样变性发生在心血管系统组织损伤区域,包括纤维化心脏瓣膜和动脉粥样斑块。3例发生在骨关节炎关节组织。值得注意的是,在子宫内膜异位囊肿壁中发生了3次,在表皮囊肿的纤维化壁中发生了4次,在疝囊中发生了1次,在皮肤溃疡的边缘发生了1次。所有沉积物都是亲血的,并表现出绿色双折射性和高锰酸盐抗性。免疫组织化学未显示对AA蛋白或免疫球蛋白 λ 或 κ 轻链的反应性。在35% 病例中检测到AP蛋白。我们的结果表明,除了通常的骨关节炎关节和受损的心脏瓣膜部位外,营养不良性淀粉样变性还会使慢性组织损伤和纤维化的其他区域复杂化,例如囊肿和溃疡壁。尽管发病机理和生化性质仍未解决,但免疫组织化学表明沉积物中不存在AA和AL蛋白,并表明涉及不同的淀粉样蛋白。

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