Kim So Hyun, Kim Jae Hwang, Gu Mi Jin
Department of Surgery, College of Medicine, Yeungnam University Daegu, Korea.
Department of Pathology, College of Medicine, Yeungnam University Daegu, Korea.
Int J Clin Exp Pathol. 2014 Mar 15;7(4):1805-8. eCollection 2014.
Amyloidosis is characterized by an extracellular deposition of insoluble fibrils. Amyloid deposition caused various clinical symptoms associated with affected organs. Secondary amyloidosis without renal involvement and chronic inflammatory conditions is rarely reported. We experienced a case of secondary intestinal amyloidosis presented with recurrent hematochezia and abdominal pain in a 54-year-old male. Sigmoidoscopy and abdominal computed tomography (CT) presented ischemic colitis and necrosis of whole colon. On microscopically, pinkish amorphous materials were infiltrated in the lamina propria and the thickened submucosal vessel walls in colon. The apple-green birefringence with polarized light on Congo red stain was demonstrated in the lamina propria and submucosal vessel walls. The deposits were positive for amyloid A and κ and negative for λ. The echocardiography and cardiac MRI findings showed infiltratives cardiomyopathy involving amyloidosis. Despite of conservative treatment, ischemic colitis and hemorrhage were aggravated and the patient expired.
淀粉样变性的特征是细胞外不溶性纤维蛋白的沉积。淀粉样沉积导致了与受影响器官相关的各种临床症状。无肾脏受累的继发性淀粉样变性和慢性炎症性疾病鲜有报道。我们遇到一例54岁男性继发性肠道淀粉样变性,表现为反复便血和腹痛。乙状结肠镜检查和腹部计算机断层扫描(CT)显示为缺血性结肠炎和全结肠坏死。显微镜下,粉红色无定形物质浸润于结肠固有层和增厚的黏膜下血管壁。刚果红染色在固有层和黏膜下血管壁呈现苹果绿双折射。沉积物对淀粉样蛋白A和κ呈阳性,对λ呈阴性。超声心动图和心脏磁共振成像结果显示浸润性心肌病合并淀粉样变性。尽管采取了保守治疗,缺血性结肠炎和出血仍加重,患者死亡。