Pruzanski W, Katz A
Can Med Assoc J. 1976 May 22;114(10):906-9.
Thirty-four patients with primary generalized amyloidosis (PGA) and 14 with multiple-myeloma-related amyloidosis (MRA) were studied. The commonest clinical manifestations in PGA were nephrotic syndrome, hepatomegaly and congestive heart failure, and in MRA, low back pain, plasmacytoma and rheumatoid-arthritis-like syndrome. Eight patients with PGA had limited clinical expression of the disease, such as involvement of only kidneys, joints, parotid glands or gastrointestinal tract; in one patient amyloidosis was limited to lymph nodes. Low serum concentrations of total protein and albumin were common. M components were detected in the serum of 91% of patients with PGA and 92% of patients with MRA: 70% of the M components in PGA and 25% of those in MRA had lambda light chains. Bence Jones proteinemia was detected in 56% of the patients with PGA and in 77% of those with MRA. The serum concentration of immunoglobulins was decreased substantially in more than two thirds of the patients with PGA. Proteinuria (greater than 250 mg/24 h) was observed in 78% of patients with PGA and in 93% of patients with MRA. Bence Jones proteinuria was noted in 75 and 77% of patients, respectively. Plasmacytic infiltration of the bone marrow was found in 90% of the patients with PGA. The mean survival time of the patients with PGA was 28 months and of those with MRA, 29 months from the time of diagnosis.
对34例原发性全身性淀粉样变性（PGA）患者和14例多发性骨髓瘤相关性淀粉样变性（MRA）患者进行了研究。PGA最常见的临床表现为肾病综合征、肝肿大和充血性心力衰竭，而MRA的常见表现为腰痛、浆细胞瘤和类风湿关节炎样综合征。8例PGA患者疾病临床表现局限，如仅累及肾脏、关节、腮腺或胃肠道；1例患者淀粉样变性仅局限于淋巴结。血清总蛋白和白蛋白浓度低很常见。91%的PGA患者和92%的MRA患者血清中检测到M成分：PGA患者中70%的M成分和MRA患者中25%的M成分具有λ轻链。56%的PGA患者和77%的MRA患者检测到本周蛋白尿。超过三分之二的PGA患者血清免疫球蛋白浓度大幅降低。78%的PGA患者和93%的MRA患者出现蛋白尿（大于250mg/24h）。分别有75%和77%的患者出现本周蛋白尿。90%的PGA患者骨髓中发现浆细胞浸润。从诊断时起，PGA患者的平均生存时间为28个月，MRA患者为29个月。
