Jimenez R E, Price D A, Pinkus G S, Owen W F, Lazarus J M, Kay J, Turner J R
Harper Hospital and Wayne State University, Detroit, Michigan 48202, USA.
Am J Surg Pathol. 1998 Jun;22(6):729-35. doi: 10.1097/00000478-199806000-00010.
Dialysis-associated beta2-microglobulin (beta2m) amyloidosis affects predominantly musculoskeletal tissue, but visceral involvement also occurs. To evaluate the clinical significance and prevalence of gastrointestinal beta2m amyloidosis, we studied hemodialysis patients admitted for gastrointestinal-related complaints. Hemodialysis patients (excluding those with non-beta2m amyloidosis) who were admitted with gastrointestinal complaints from 1984 to 1994 were identified. Gastrointestinal tissues from patients with available autopsy or surgical specimens were examined using hematoxylin and eosin stain, Congo red stain, and beta2m immunostain. Each case was evaluated independently by two pathologists and scored for quantity and location of beta2m amyloid and associated pathology. Of 24 patients, eight (four men and 4 women) had beta2m amyloid deposits within the gastrointestinal tract. Acute clinical presentation ranged from abdominal pain to gastrointestinal bleeding and was not significantly different for patients with or without gastrointestinal beta2m amyloid deposits. However, the mean time on dialysis of 15.3 +/- 5.7 years (range 6-24 years) for patients with gastrointestinal beta2m amyloidosis was significantly greater than that of patients without gastrointestinal beta2m amyloidosis (10.5 +/- 7.0 years, range <1 to 22 years, p < 0.05). Vascular histopathology ranged from mild focal thickening of vessel walls to massive vascular beta2m amyloid deposition with thrombosis. Extravascular beta2m amyloid ranged from mild to severe with marked expansion of the submucosa. Mucosal pathology ranged from none to severe ulceration. The degree of beta2m amyloid and the associated pathology tended to increase in severity with time on dialysis. Gastrointestinal beta2m amyloid deposition is an underappreciated complication of chronic hemodialysis that is significantly associated with increased time on dialysis. Gastrointestinal beta2m amyloidosis should be considered in any patient on hemodialysis 10 years or more who has gastrointestinal symptoms and can be identified in resection specimens as well as some biopsy specimens. Congo red stain and beta2m immunostains may be necessary for sensitive histopathologic evaluation of gastrointestinal beta2m amyloidosis.
透析相关的β2微球蛋白（β2m）淀粉样变性主要累及肌肉骨骼组织，但也会出现内脏受累情况。为评估胃肠道β2m淀粉样变性的临床意义和患病率，我们对因胃肠道相关症状入院的血液透析患者进行了研究。确定了1984年至1994年期间因胃肠道症状入院的血液透析患者（不包括非β2m淀粉样变性患者）。对有可用尸检或手术标本的患者的胃肠道组织进行苏木精-伊红染色、刚果红染色和β2m免疫染色检查。由两名病理学家独立评估每个病例，并对β2m淀粉样蛋白的数量和位置以及相关病理进行评分。在24例患者中，8例（4名男性和4名女性）胃肠道内有β2m淀粉样蛋白沉积。急性临床表现从腹痛到胃肠道出血不等，有或没有胃肠道β2m淀粉样蛋白沉积的患者之间无显著差异。然而，胃肠道β2m淀粉样变性患者的平均透析时间为15.3±5.7年（范围6 - 24年），显著长于无胃肠道β2m淀粉样变性的患者（10.5±7.0年，范围<1至22年，p<0.05）。血管组织病理学表现从血管壁轻度局灶性增厚到伴有血栓形成的大量血管β2m淀粉样蛋白沉积。血管外β2m淀粉样蛋白从轻度到重度不等，黏膜下层有明显扩张。黏膜病理从无到严重溃疡不等。β2m淀粉样蛋白的程度和相关病理往往随着透析时间的延长而加重。胃肠道β2m淀粉样蛋白沉积是慢性血液透析中一种未得到充分认识的并发症，与透析时间延长显著相关。对于任何透析10年或更长时间且有胃肠道症状的血液透析患者，都应考虑胃肠道β2m淀粉样变性，并且在切除标本以及一些活检标本中都可以识别出来。刚果红染色和β2m免疫染色可能是对胃肠道β2m淀粉样变性进行敏感组织病理学评估所必需的。
