Montseny J J, Kleinknecht D, Meyrier A, Vanhille P, Simon P, Pruna A, Eladari D
Service de Néphrologie et Réanimation Polyvalente, Centre Hospitalier, Montreuil, France.
Nephrol Dial Transplant. 1998 Jun;13(6):1438-45. doi: 10.1093/ndt/13.6.1438.
The prognosis of monoclonal gammopathies with multiple myeloma and renal involvement is poor, and the indication for renal replacement therapy is controversial. Few studies address the value of renal histology for determining prognosis according to initial pathology findings.
We studied the course of 118 patients with multiple myeloma according to renal biopsy lesions. The monoclonal component was identified and quantified in serum and urine. Tumor cell mass was classified as stage 1, 2 or 3, according to Durie and Salmon. End-points were death, or survival on dialysis, or serum creatinine level at last examination.
Renal biopsy showed myeloma kidney in 48 cases (41%), AL-amyloidosis in 35 (30%), light chain deposit disease in 22 (19%), chronic tubulointerstitial nephritis in 12 (10%) and cryoglobulinaemic kidney with multiple myeloma in 1. Maintenance haemodialysis was required in 46 patients (39%), earlier (P<0.0001) in myeloma kidney (mean: 3 months after diagnosis) than in AL-amyloidosis (mean: 15 months) and light chain deposit disease (mean: 18 months). Median survival was 12 months in myeloma kidney, 24 months in AL-amyloidosis and 48 months in light chain deposit disease. Dialysis increased survival in light chain deposit disease, in contrast with myeloma kidney and AL-amyloidosis patients whose survival was shorter when dialysed. The main cause of death during first year of dialysis was cardiac involvement in AL-amyloidosis, and sepsis or cardiac insufficiency in myeloma kidney. There was a trend to increased survival with multidrug chemotherapy which seemed to slow progression to end-stage renal failure. At last follow-up (median: 12 months, range 1-297), 65 (55%) patients had died. By multivariate analysis, independent predictors of survival were: age < 70, serum creatinine < or = 300 micromol/l, and serum calcium < or = 2.5 mmol/l.
Initial renal biopsy helps predict prognosis in patients with multiple myeloma and renal involvement. Maintenance haemodialysis is a reasonable indication in light chain deposit disease and AL-amyloidosis, especially in patients aged < 70. Multidrug therapy tends to prolong survival and slow progression to end-stage renal disease.
合并多发性骨髓瘤及肾脏受累的单克隆丙种球蛋白病预后较差，肾脏替代治疗的指征存在争议。很少有研究探讨根据初始病理结果判断肾脏组织学对预后的价值。
我们根据肾脏活检病变研究了118例多发性骨髓瘤患者的病程。对血清和尿液中的单克隆成分进行鉴定和定量。根据Durie和Salmon标准，将肿瘤细胞量分为1、2或3期。观察终点为死亡、透析生存或最后一次检查时的血清肌酐水平。
肾脏活检显示48例（41%）为骨髓瘤肾病，35例（30%）为AL型淀粉样变性，22例（19%）为轻链沉积病，12例（10%）为慢性肾小管间质性肾炎，1例为合并多发性骨髓瘤的冷球蛋白血症性肾病。46例患者（39%）需要维持性血液透析，骨髓瘤肾病患者开始透析的时间更早（P<0.0001）（平均：诊断后3个月），而AL型淀粉样变性患者为（平均：15个月），轻链沉积病患者为（平均：18个月）。骨髓瘤肾病患者的中位生存期为12个月，AL型淀粉样变性患者为24个月，轻链沉积病患者为48个月。与骨髓瘤肾病和AL型淀粉样变性患者透析后生存期缩短相反，透析可提高轻链沉积病患者的生存率。透析第一年的主要死亡原因，在AL型淀粉样变性患者中是心脏受累，在骨髓瘤肾病患者中是败血症或心功能不全。多药化疗有提高生存率的趋势，似乎可减缓向终末期肾衰竭的进展。在最后一次随访时（中位时间：12个月，范围1 - 297个月），65例（55%）患者死亡。多因素分析显示，生存的独立预测因素为：年龄<70岁、血清肌酐≤300μmol/L和血清钙≤2.5mmol/L。
初始肾脏活检有助于预测合并多发性骨髓瘤及肾脏受累患者的预后。维持性血液透析对于轻链沉积病和AL型淀粉样变性患者是合理的指征，尤其是年龄<70岁的患者。多药治疗倾向于延长生存期并减缓向终末期肾病的进展。
