多发性骨髓瘤肾脏病变的研究进展

 多发性骨髓瘤（MM）患者肾损害谱包括"骨髓瘤肾"或管型肾病、轻链淀粉样变、单克隆免疫球蛋白沉积病、冷球蛋白血性肾小球肾炎和增生性肾小球肾炎等。MM肾脏病患者肾活组织病理检查研究显示,管型肾病占40 %～63 %,肾轻链沉积病（LCDD）占19 %～26 %,淀粉样变占7 %～30 %,冷球蛋白肾病＜1 %。管型肾病、MIDD和淀粉样变的肾病理学不同。骨髓瘤相关肾病的肾外表现较多样,进展为肾功能不全和末期肾病（ESRD）发生率高,并发症增加,死亡率显著升高,合并肾病的MM患者生存期和肾生存期短。治疗包括MM化疗、血液透析和自体造血干细胞移植（ASCT）等,ASCT有血液学反应和肾反应者的生存率明显改善,成功的ASCT给更多的患者以肾和其他脏器移植的机会。

Progress of research on renal disease in multiple myeloma

The spectrum of renal lesions that was seen in patients with myeloma include "myeloma kidney", or cast nephropathy; light chain (AL) amyloidosis; monoclonal Ig deposition disease(MIDD); and less frequently, cryoglobulinemic glomerulonephritis and proliferative glomerulonephritis.ln native renal biopsy studies of patients with myeloma and renal disease, 40 % to 63 % had east nephropathy, 19 % to 26 % had light-chain deposition disease, 7 % to 30 % had amyioidosis, and 1% had eryoglobulinemie renal disease. The renal pathology of cast nephropathy, MIDD, and amyloidosis was diverse.Extrarenal manifestations of MIDD, as with amyloidosis, were frequent, the incidence of renal insufficiency and ESRD was high.The morbidity and mortality were a significant increase.Patient and renal survival were significantly worse in patients with coexisting nephropathy.Therapy of renal lesions with myeloma was similar to that for multiple myeloma and consisted of chemotherapy alone,and treated renal insufficiency with hemodialysis and peritoneal dialysis, and used of high-dosage chemotherapy followed by ASCT to reduced the level of light-chain production.