Zerbini Cristiano A F, Anderson Jennifer J, Kane Kelly A, Ju Shyr-Te, Campistol Jose M, Simms Robert W, Cohen Alan S, Skinner Martha
Thorndike Memorial Laboratory, Division of Medicine, Boston City Hospital, Arthritis Center, Boston University School of Medicine, Boston, Massachusetts, USA.
Amyloid. 2002 Dec;9(4):242-6. doi: 10.3109/13506120209114100.
To study the relation between beta 2 microglobulin (beta 2M) and survival in AL amyloidosis, we measured the serum level of beta 2M in 80 patients with AL amyloidosis diagnosed within 1 year of evaluation, who had received no therapy. Patients had a median age of 61 years and 52% were male. Major clinical manifestations were renal disease in 25 patients (31%), cardiomyopathy in 23 patients (29%), and neuropathy or other organ involvement in 32 patients (41%). The beta 2M level, measured by an ELISA assay in serum samples collected at the time of evaluation, ranged from 1.69 to 10 mg/ml (mean = 4.57); in 56% of the patients beta 2M > 4 mg/ml. The patients with a beta 2M < or = 4 mg/ml had serum creatinine levels lower than those with beta 2M > 4 (1.43 vs 2.67 mg/dl; p = 0.02). Survival from study entry was analyzed overall by the level of beta 2M, adjusting for creatinine level and clinical stratum. We found the beta 2M level to be predictive of survival (median survival 16.1 months for beta 2M < or = 4 mg/ml vs 8.0 months for beta 2M > 4 mg/ml, p = 0.044). Thus a beta 2M level less than 4 mg/ml indicated a longer time of survival.
为研究β2微球蛋白（β2M）与AL淀粉样变性患者生存率之间的关系，我们检测了80例在评估后1年内确诊且未接受过治疗的AL淀粉样变性患者的血清β2M水平。患者的中位年龄为61岁，52%为男性。主要临床表现为：25例患者（31%）有肾脏疾病，23例患者（29%）有心肌病，32例患者（41%）有神经病变或其他器官受累。通过ELISA法检测评估时采集的血清样本中的β2M水平，范围为1.69至10mg/ml（平均 = 4.57）；56%的患者β2M>4mg/ml。β2M≤4mg/ml的患者血清肌酐水平低于β2M>4mg/ml的患者（1.43 vs 2.67mg/dl；p = 0.02）。根据β2M水平对研究入组后的生存率进行总体分析，并对肌酐水平和临床分层进行校正。我们发现β2M水平可预测生存率（β2M≤4mg/ml的患者中位生存期为16.1个月，而β2M>4mg/ml的患者为8.0个月，p = 0.044）。因此，β2M水平低于4mg/ml表明生存时间更长。
