移植肾轻链沉积病二例并文献复习

Light chain deposition disease after renal transplantation: a report of 2 cases and a review of literature

目的探讨在肾移植术后容易复发的轻链沉积病受者临床、病理、治疗及转归资料。方法对2例移植肾轻链沉积病受者的临床、病理、治疗及转归进行分析并复习相关文献。1例男,49岁,原发病不明,肾移植术后24个月,尿检异常,血肌酐升高,无贫血,移植肾穿刺活检提示轻链沉积病。另1例男,38岁,原发病为轻链沉积病,肾移植术后15个月,尿蛋白临界值,镜下血尿,血肌酐升高,无贫血及高血压,移植肾穿刺活检提示复发性轻链沉积病。结果1例予硼替佐米联合地塞米松治疗,随访期间血肌酐下降。1例予硼替佐米联合地塞米松治疗。随访期间合并脓毒症及肺部感染,肾功能损害进展。结论肾移植术后轻链沉积病少见,进展快,预后差,肾移植术前及术后及早完善血尿游离轻链及免疫固定电泳有助于早期诊断,硼替佐米治疗移植肾轻链沉积病疗效有待进一步观察。

Objective To report for the first time the clinicopathologic characteristics of two patients with light chain deposition disease after renal transplantation.Methods The clinicopathologic features were analyzed for two patients with light chain deposition disease and the related literature was reviewed.Case 1 was a 49-year-old male with unknown primary disease presenting with abnormal urine test and elevated serum creatinine at 24 months after kidney transplantation.Renal allograft biopsy hinted at light chain deposition disease.Case 2 was a 38-year-old male with light chain deposition disease in native kidneys presenting with proteinuria,microscopic hematuria and elevated serum creatinine at 15 months after kidney transplantation.Renal allograft biopsy supported recurrent light chain deposition disease.Results 1 case after bortezomib and dexamethasone dosing,serum creatinine decreased during follow-ups.1 case received bortezomib and dexamethasone.However,sepsis and pulmonary infections developed and allograft function deteriorated.Conclusions Light chain deposition disease after renal transplantation is a rare disorder with a rapid progression and a poor prognosis.Early detection of free light chain in blood and urine through immune fixed electrophoresis is conducive to an early diagnosis.The efficacy of bortezomib is to be furthered examined.