Department of Pathology and Laboratory Medicine, Temple University Hospital, Philadelphia, PA 19140, USA.
Amyloid. 2011 Dec;18(4):245-8. doi: 10.3109/13506129.2011.623738. Epub 2011 Oct 20.
We report a case of a 41-year-old African-American male paraplegic for more than 20 years, who had recurrent infections, renal failure with total urine protein of 840 mg/dL on urine protein electrophoresis and adrenal cortical insufficiency. He died suddenly of massive gastrointestinal (GI) hemorrhage. Autopsy showed clinically undiagnosed systemic amyloidosis involving the kidneys, adrenal cortices, spleen and small blood vessels of most organs and tissues, including those of the gastrointestinal tract. The history and autopsy findings indicated secondary or amyloid A (AA) amyloidosis. Paraplegia was one of the most common causes of secondary amyloidosis decades ago, but has now become unusual in patients with AA amyloidosis. Extensive involvement of GI small vessels was the most likely cause of fatal bleeding. GI amyloid previously has been shown to cause hemorrhage, but a fatal case has not yet been described.
我们报告了一例 41 岁的非洲裔美国男性截瘫患者，他已经截瘫超过 20 年，反复发生感染，肾功能衰竭，尿蛋白电泳总蛋白为 840mg/dL，且伴有肾上腺皮质功能不全。他因大量胃肠道（GI）出血突然死亡。尸检显示，临床上未诊断出的系统性淀粉样变性累及肾脏、肾上腺皮质、脾脏和大多数器官和组织的小血管，包括胃肠道。病史和尸检结果提示为继发性或淀粉样蛋白 A（AA）淀粉样变性。截瘫是几十年前继发性淀粉样变性最常见的原因之一，但现在在 AA 淀粉样变性患者中已不常见。胃肠道小血管的广泛受累极有可能是致命性出血的原因。胃肠道淀粉样变性以前曾被证明会引起出血，但尚未有致命病例的报道。
