Kanoh T, Inoue M, Okuma M
Department of Internal Medicine, Faculty of Medicine, Kyoto University.
Rinsho Ketsueki. 1993 Aug;34(8):952-6.
Recently, we have encountered an unusual case of "monoclonal gammopathy of undetermined significance (MGUS)" in which some clinical features indicative of primary and amyloidosis (PA) were observed two years after the diagnosis of MGUS. The patient, born in 1925, was diagnosed of having MGUS (IgG-lambda type M-component level; 1 g/dl) in June 1990. The following clinical features occurred in close succession within five months after a stable course of two years: general malaise, abdominal, distension, pretibial edema and facial puffiness, ecchymoses on the chest wall, and dyspnea on effort. The biopsy specimens from the skin and gastric mucosa revealed amyloid deposition. The M-component levels in the serum and urine as well as the number of bone marrow plasma cells remained unchanged. The various kinds of laboratory examinations indicated that systemic amyloidosis rapidly developed within five months. It seems quite likely that the heart, liver, and spleen may be affected with amyloidosis. It is noteworthy that PA can occur without an increase in the serum M-component level at any time after the diagnosis of MGUS. An early diagnosis of PA as well as multiple myeloma should be kept in mind in the follow-up study of patients with MGUS.
最近，我们遇到了一例不寻常的意义未明的单克隆丙种球蛋白病（MGUS），在MGUS诊断两年后观察到了一些提示原发性淀粉样变性（PA）的临床特征。该患者出生于1925年，1990年6月被诊断为患有MGUS（IgG-λ型M蛋白水平；1g/dl）。在经历了两年的稳定病程后，以下临床特征在五个月内相继出现：全身不适、腹胀、胫前水肿和面部浮肿、胸壁瘀斑以及活动后呼吸困难。皮肤和胃黏膜活检标本显示有淀粉样沉积。血清和尿液中的M蛋白水平以及骨髓浆细胞数量保持不变。各种实验室检查表明，系统性淀粉样变性在五个月内迅速发展。心脏、肝脏和脾脏很可能受到淀粉样变性的影响。值得注意的是，在MGUS诊断后的任何时候，PA都可能在血清M蛋白水平未升高的情况下发生。在对MGUS患者进行随访研究时，应牢记PA以及多发性骨髓瘤的早期诊断。
