Scherwitz C, Linke R P, Schmitt F J
Hautarzt. 1986 Jun;37(6):343-6.
A 73-year-old patient complained of increasing macroglossia and symmetrical enlargement of the submandibular region. A tongue biopsy showed typical deposits of amyloid by Congo red staining and polarization microscopy. Similar deposits were found in biopsy specimens of the submaxillary gland and the rectum. Electron microscopy revealed typical amyloid fibrils. With the indirect immunoperoxidase technique the amyloid was classified as being of immunoglobulin kappa-light-chain origin (A-kappa), because only anti-A kappa, but not anti-A lambda (several antisera of non-overlapping specificity), anti-ASc1 or anti-AF antisera were found to be reactive. Correspondingly, Bence Jones protein of the kappa type was found in the urine. In the bone marrow there was an increase in plasma cells of up to 10%. Cytostatic therapy with melphalan and methylprednisolone seemed to stop the progression of amyloid deposition.
一名73岁患者主诉巨舌症加重且下颌下区域对称性肿大。舌活检经刚果红染色和偏振显微镜检查显示有典型的淀粉样蛋白沉积。在颌下腺和直肠活检标本中也发现了类似的沉积物。电子显微镜显示有典型的淀粉样纤维。采用间接免疫过氧化物酶技术，该淀粉样蛋白被归类为免疫球蛋白κ轻链来源（A-κ），因为仅发现抗Aκ，而非抗Aλ（几种具有非重叠特异性的抗血清）、抗ASc1或抗AF抗血清具有反应性。相应地，在尿液中发现了κ型本-周蛋白。骨髓中浆细胞增加至10%。用美法仑和甲基泼尼松龙进行的细胞抑制治疗似乎阻止了淀粉样蛋白沉积的进展。
