Song-su K, Mitarai T, Tamura H, Nagasawa R, Hirose S, Itakura Y, Sagara T, Isoda K
Fourth Department of Internal Medicine, Saitama Medical Center, Saitama Medical School.
Nihon Jinzo Gakkai Shi. 1992 Jun;34(6):739-42.
A 67-year-old man was hospitalized with a diagnosis of nephrotic syndrome. Physical findings at admission were generalized edema and macroglossia. Urinalysis showed massive proteinuria, + +occult blood, and granular and broad casts. Ig A lambda monoclonal gammopathy was noted in the serum. There was no evidence of myeloma in the bone marrow aspirate, scintigram or X-ray of the bone. A biopsy specimen of the kidney showed massive deposits of structureless material in the glomeruli. Marked cell infiltration was also observed in the interstitium. Multinucleated giant cells were occasionally seen in the Bowman's capsules and the interstitium. There were reactive changes in the Bowman's capsule adjacent to the giant cell. The deposits were proved to be amyloid by positive staining with Congo red and apple-green birefringence by polarized light. In addition, microfibrills seen on electron microscopy displayed deposits. Amyloid depositions were observed in other tissues such as gingiva, skin and tongue. Staining of amyloid with Congo red was resistant to potassium permanganate, and amyloid was positively stained with lambda-light chain of immunoglobulin. These findings indicated that the patient had primary amyloidosis. Infiltration of the multinucleated giant cell has been reported only in patients with familial amyloidosis and secondary amyloidosis associated with rheumatoid arthritis. To our knowledge the present case is a first report of the giant cell infiltration in a Bowman's capsule in primary amyloidosis.
一名67岁男性因肾病综合征诊断入院。入院时体格检查发现全身水肿和巨舌。尿液分析显示大量蛋白尿、++潜血以及颗粒管型和宽大管型。血清中发现IgA λ单克隆丙种球蛋白病。骨髓穿刺、骨闪烁扫描或骨骼X线检查均未发现骨髓瘤证据。肾脏活检标本显示肾小球内有无结构物质的大量沉积。间质中也观察到明显的细胞浸润。在鲍曼囊和间质中偶尔可见多核巨细胞。与巨细胞相邻的鲍曼囊有反应性改变。经刚果红阳性染色及偏振光下苹果绿双折射证实沉积物为淀粉样物质。此外，电子显微镜下可见的微原纤维显示有沉积物。在牙龈、皮肤和舌等其他组织中也观察到淀粉样沉积。淀粉样物质刚果红染色对高锰酸钾有抗性，且淀粉样物质经免疫球蛋白λ轻链阳性染色。这些发现表明该患者患有原发性淀粉样变性。多核巨细胞浸润仅在家族性淀粉样变性和与类风湿关节炎相关的继发性淀粉样变性患者中报道过。据我们所知，本病例是原发性淀粉样变性中鲍曼囊内巨细胞浸润的首例报告。
