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[胃AL淀粉样变病例中的致死性Dieulafoy型溃疡]

[Fatal Dieulafoy's type ulcer in a case of gastric AL-amyloidosis].

作者信息

Youssef Nelly, Carbonell Nicolas, Bonte Hélène, Serfaty Lawrence, Poupon Raoul, Fléjou Jean-François

机构信息

Service d'Anatomie Pathologique, Hôpital Necker-Enfants Malades, 149 rue de Sèvres 75743 Paris Cedex 15.

出版信息

Ann Pathol. 2004 Jun;24(3):256-8. doi: 10.1016/s0242-6498(04)93960-0.

Abstract

Dieulafoy's ulcer is a particular form of gastric ulcer confined to a persistent caliber artery and may lead to severe hemorrhage. We report a case of fatal gastric bleeding in a woman with benign biclonal gammapathy. Autopsy found a typical Dieulafoy's ulcer centered by a persistent caliber artery which wall was thickened by AL-amyloid deposits. Amyloidosis involved the gastric wall, but also middle caliber arteries of the liver, the lung, the pancreas, the kidney and the myocardium. AL-amyloidosis is a rare and late complication of monoclonal gammapathy and may be asymptomatic. Pathogenesis of Dieulafoy's ulcer remains unclear. In our case, local ischemia may have facilitated gastric ulceration, and amyloid deposits may have contributed to arterial rupture.

摘要

迪厄拉富瓦溃疡是一种特殊类型的胃溃疡,局限于一条持续性管径动脉,可能导致严重出血。我们报告一例患有良性双克隆丙种球蛋白病的女性发生致命性胃出血的病例。尸检发现一个典型的迪厄拉富瓦溃疡,其中心为一条持续性管径动脉,动脉壁因AL淀粉样蛋白沉积而增厚。淀粉样变性累及胃壁,但也累及肝脏、肺、胰腺、肾脏和心肌的中等管径动脉。AL淀粉样变性是单克隆丙种球蛋白病的一种罕见且晚期并发症,可能无症状。迪厄拉富瓦溃疡的发病机制仍不清楚。在我们的病例中,局部缺血可能促进了胃溃疡形成,而淀粉样蛋白沉积可能导致了动脉破裂。

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