Gertz Morie A, Kyle Robert A
Dysproteinemia Clinic and the Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 55905, USA.
Semin Oncol. 2003 Apr;30(2):325-8. doi: 10.1053/sonc.2003.50060.
We sought to review outcomes of patients who have immunoglobulin light-chain amyloidosis of the IgM type. Fifty patients with a serum IgM monoclonal protein and biopsy-proven amyloid were evaluated. The percentages of patients presenting with cardiac, renal, hepatic, and pulmonary amyloid were 44%, 32%, 14%, and 10%, respectively. Forty-two percent had an M-protein spike in the serum greater than 1.5 g/dL, and 14% had an IgM peak greater than 3 g/dL. Amyloidosis was diagnosed easily by biopsies of the subcutaneous fat, rectum, and bone marrow, which demonstrated deposits in 84%, 72%, and 50%, respectively. The median survival of all patients was 24.6 months. Fifty-three percent of all deaths were due to cardiac amyloidosis. Twelve percent died of respiratory failure, and 7% each died of macroglobulinemia, hepatic failure, and renal failure. Of the 22 patients with amyloid cardiomyopathy, the median survival was 11.1 months and fewer than 10% survived 5 years. Of the 28 patients without amyloid cardiomyopathy at diagnosis, the median survival was 27 months, with approximately 30% surviving 5 years. We conclude that the presence of amyloid cardiomyopathy and an increased creatinine concentration had the greatest impact on survival.
我们试图回顾患有IgM型免疫球蛋白轻链淀粉样变性患者的预后情况。对50例血清IgM单克隆蛋白且经活检证实为淀粉样变性的患者进行了评估。出现心脏、肾脏、肝脏和肺部淀粉样变性的患者百分比分别为44%、32%、14%和10%。42%的患者血清M蛋白峰高于1.5 g/dL，14%的患者IgM峰高于3 g/dL。通过皮下脂肪、直肠和骨髓活检很容易诊断出淀粉样变性，其显示沉积物的比例分别为84%、72%和50%。所有患者的中位生存期为24.6个月。所有死亡病例中有53%是由于心脏淀粉样变性。12%死于呼吸衰竭，各有7%死于巨球蛋白血症、肝衰竭和肾衰竭。在22例患有淀粉样心肌病的患者中，中位生存期为11.1个月，不到10%的患者存活5年。在诊断时无淀粉样心肌病的28例患者中，中位生存期为27个月，约30%的患者存活5年。我们得出结论，淀粉样心肌病的存在和肌酐浓度升高对生存期影响最大。
