Sethi D, Hutchison A J, Cary N R, Brown E A, Curtis J R, Woodrow D F, Gower P E
Department of Medicine, Charing Cross Hospital, London, UK.
Nephron. 1990;55(3):312-5. doi: 10.1159/000185981.
A 48-year-old male on cuprophane haemodialysis for 18 years, with a history of dialysis arthropathy and recurrent carpal tunnel syndrome developed macroglossia and bilateral buttock tumoral masses. The tongue and buttock masses were biopsied. Histology of both biopsies showed amyloid deposits of the beta 2-microglobulin (B2M) variety. Amyloidomas in the gluteal region and macroglossia have not been previously described in amyloid derived from B2M. These findings suggest that systemic B2M amyloidosis can have a similar tissue distribution to AL amyloidosis. This case also stresses the importance of inspection of the tongue, and palpation of the gluteal region for masses, in the assessment of patients with dialysis arthropathy.
一名48岁男性，使用铜仿膜进行血液透析18年，有透析性关节病和复发性腕管综合征病史，出现巨舌和双侧臀部肿瘤性肿块。对舌部和臀部肿块进行了活检。两次活检的组织学检查均显示为β2-微球蛋白（B2M）型淀粉样沉积物。源自B2M的淀粉样变中，此前尚未有臀区淀粉样瘤和巨舌的相关描述。这些发现表明，全身性B2M淀粉样变的组织分布可能与AL淀粉样变相似。该病例还强调了在评估透析性关节病患者时，检查舌部和触诊臀部有无肿块的重要性。
