Díaz Del Arco C, Fernández Aceñero M J
Department of Surgical Patholology, Hospital Clínico San Carlos, Madrid, Spain.
Department of Surgical Patholology, Hospital Clínico San Carlos, Madrid, Spain.
Arab J Gastroenterol. 2018 Jun;19(2):96-99. doi: 10.1016/j.ajg.2018.03.003. Epub 2018 May 24.
Gastrointestinal involvement is frequent in systemic amyloidosis. However, amyloidosis can rarely be confined to the gastrointestinal tract or appear as a tumour mass. There have been few reports describing amyloid globular deposits in a variety of locations, as opposed to the usual linear ones. We herein report a rare case of globular amyloidosis involving the large bowel, which to the best of our knowledge is the second reported in the world literature. A 74-year-old man consulted on anaemia. Endoscopy showed ulcerative lesions in the left colon, which were biopsied and diagnosed as ischemic colitis. Under light microscopy, we found globular discrete deposits in the lamina propria which were Congo red-positive and resistant to permanganate digestion. Histopathological diagnosis was globular amyloidosis with AL deposits. The patient underwent further studies, including a haematologic evaluation that discarded systemic involvement. Globular amyloidosis seems to be a rare morphologic type of amyloidosis, but not a distinct entity. Its etiology, pathogenesis and relationship with patient prognosis and disease severity remain largely unknown. When amyloid deposits are confined to the gastrointestinal tract, systemic therapy can be avoided and patients should only be followed periodically. Immunohistochemical classification and clinical correlation are essential to rule out systemic amyloidosis.
胃肠道受累在系统性淀粉样变性中很常见。然而，淀粉样变性很少局限于胃肠道或表现为肿瘤肿块。与通常的线性沉积不同，很少有报告描述各种部位的淀粉样球状沉积。我们在此报告一例罕见的累及大肠的球状淀粉样变性病例，据我们所知，这是世界文献中第二例报道。一名74岁男性因贫血前来就诊。内镜检查显示左半结肠有溃疡性病变，对病变进行活检并诊断为缺血性结肠炎。在光学显微镜下，我们在固有层发现了球状离散沉积物，刚果红染色阳性且对高锰酸钾消化有抗性。组织病理学诊断为伴有AL沉积的球状淀粉样变性。患者接受了进一步检查，包括血液学评估，排除了系统性受累。球状淀粉样变性似乎是一种罕见的淀粉样变性形态类型，但并非一个独特的实体。其病因、发病机制以及与患者预后和疾病严重程度的关系在很大程度上仍不清楚。当淀粉样沉积局限于胃肠道时，可以避免全身治疗，仅需定期对患者进行随访。免疫组织化学分类和临床相关性对于排除系统性淀粉样变性至关重要。
