多发性骨髓瘤管型肾病和肾淀粉样变性的临床特征及预后分析

Clinical features and prognosis of multiple myeloma cast nephropathy and amyloidosis

目的比较肾活检诊为多发性骨髓瘤管型肾病(MM-MCN)及肾淀粉样变性(MM-AL)的临床特点,同时分析MM-MCN患者预后。方法收集近5年北京协和医院首次确诊多发性骨髓瘤(MM)、肾活检病理为MM-MCN和MM-AL患者的临床资料,观察MCN患者化疗后的血液学及肾脏反应。结果MM-MCN组(n=10)出现肾损害时病程短于MM-AL组(n=13)(P<0.05),肾病综合征的比例低于MM-AL组(P<0.05);血红蛋白水平低于MM-AL组(P<0.001),血肌酐水平高于MM-AL组(P<0.001)。MM-MCN组骨髓浆细胞比例、24 h尿轻链水平均高于MM-AL组(P<0.05)。10例MM-MCN患者均表现为急性肾损伤,经硼替佐米或沙利度胺为基础的化疗治疗后,呈较高的血液和肾脏反应率(62.5%和75.0%)。结论MM患者如果表现为肾病综合征、轻中度肾功能损害、肾病病程较长、较低水平的尿轻链及骨髓浆细胞比例者可能合并有MM-AL,应尽早肾活检明确诊断。MM-MCN患者即使出现严重肾功能损害可接受硼替佐米或沙利度胺为基础的化疗。

Objective To compare clinical features of multiple myeloma cast nephropathy(MM-MCN)and myeloma amyloidosis(MM-AL)and to evaluate the prognosis of MM-MCN.Methods The clinical characteristics of patients biopsy-proved MM-MCN and MM-AL over 5 years at Peking Union Medical College Hospital were collected.The hematologic and renal responses of MM-MCN patients after chemotherapy were also reviewed.Results The duration of renal involvement was shorter in MM-MCN(n=10)than that in MM-AL(n=13)(P<0.05).The nephrotic syndrome of MM-MCN was less frequent than that of MM-AL(P<0.05).The hemoglobin level was lower in MM-MCN than those in MM-AL(P<0.001)while the serum creatinine level was higher in the former than those in the latter(P<0.001).The percentage of bone marrow plama cell and 24 h urine light chain level in MM-MCN group were higher than those in MM-AL group(both P<0.05).Ten MM-MCN patients all manifested acute renal injury at the time of biopsy and showed rather high hematologic and renal response rates after treatment regimen based on bortezomib or thalidomide(62.5%and 75.0%respectively).Conclusions MM patients showed nephrotic syndrome,mild to moderate renal dysfunction,longer duration of renal damage,lower urine light chain level or less percentage of bone marrow plama cell are prone to have amyloidosis,and the renal biopsy is indicated.The MM-MCN patients with deteriorated renal function may be benefited from chemotherapy based on bortezomib or thalidomide.