Spanti D, Marcantoni C, Di Landro D
U.O. di Nefrologia e Dialisi, Azienda Ospedaliera Cannizzaro, Catania.
G Ital Nefrol. 2005 Nov-Dec;22 Suppl 33:S39-45.
Several renal diseases are associated with the dysproteinemias, and their pathogenesis is related to paraprotein deposits in the kidney: light chains can affect the kidney by a direct toxic effect on tubular cells, or by intratubular or tissue precipitation. Multiple myeloma (MM) is the most prevalent dysproteinemia, and the spectrum of associated renal diseases includes myeloma kidney (cast nephropathy), amyloidosis and monoclonal immunoglobulin deposition disease (MIDD). Renal failure is seen in approximately 50% of patients with MM at diagnosis, most frequently attributed to myeloma kidney. Renal function can recover in more than half the patients by prompt rehydration with intravenous fluids, to achieve a urine flow of >3 l/day, and by treating the hypercalcemia. Plasma exchange in combination with corticosteroids is suggested in patients with rapidly progressive renal failure. When renal failure is associated with MIDD or amyloidosis, renal function recovery is reduced to 10%, and patient survival is related to the entity of extrarenal tissue distribution of paraprotein deposits. Dialysis should be offered to patients with end-stage renal disease. High dose chemotherapy and autologous stem cells transplantion (SCT) is recommended in patients who do not have severe co-morbidities.
几种肾脏疾病与蛋白异常血症相关，其发病机制与副蛋白在肾脏中的沉积有关：轻链可通过对肾小管细胞的直接毒性作用，或通过肾小管内或组织沉淀来影响肾脏。多发性骨髓瘤（MM）是最常见的蛋白异常血症，与之相关的肾脏疾病谱包括骨髓瘤肾病（管型肾病）、淀粉样变性和单克隆免疫球蛋白沉积病（MIDD）。约50%的MM患者在诊断时出现肾衰竭，最常见的原因是骨髓瘤肾病。通过静脉补液迅速补液以达到每日尿量>3升，并治疗高钙血症，超过一半的患者肾功能可恢复。对于快速进展性肾衰竭患者，建议采用血浆置换联合皮质类固醇治疗。当肾衰竭与MIDD或淀粉样变性相关时，肾功能恢复率降至10%，患者生存率与副蛋白沉积的肾外组织分布情况有关。终末期肾病患者应接受透析治疗。对于没有严重合并症的患者，建议进行高剂量化疗和自体干细胞移植（SCT）。
