缺乏肾脏单克隆丙种球蛋白病(MGRS)患者的肾移植结果数据。
MGRS患者的病例系列,其中一些人在肾移植前接受了克隆导向治疗.
从1987年到2016年,28例接受肾移植的患者诊断为MGRS相关病变,包括轻链沉积病(LCDD)。C3肾小球病伴单克隆丙种球蛋白病(C3G-MG),和轻链近端肾小管病(LCPT)。
在19例LCDD患者中,10例在肾移植前治疗,9例初治。在接受治疗的LCDD患者中,3(30%)经历了组织学复发,2(20%)移植失败,2人(20%)在中位70次随访期间死亡(范围,移植后3-162)个月。在未治疗的LCDD组中,8例(89%)组织学复发,6例(67%)移植失败,在中位60次随访期间,有4例(44%)患者死亡(范围,35-117)个月。在移植前完全缓解的5名患者中,没有人死亡,只有1人经历了移植失败,移植后162个月。5例C3G-MG患者中,3是移植前的治疗。移植前接受治疗的两名患者均有组织学复发,1例移植失败并死亡。在3例未经治疗的C3G-MG患者中,组织学复发发生在所有,移植物丢失和死亡分别为2和1。在LCPT组(n=4)中,在移植前未接受克隆定向治疗的所有3例患者中观察到组织学复发。其中两个病人死亡,1具有功能正常的肾脏。在移植前接受治疗的1例LCPT患者没有组织学复发或移植物丢失,并存活。
小样本量,不规范的临床管理,回顾性设计。
在肾移植后所有MGRS相关病变中复发非常常见。实现完全的血液学反应可以降低复发的风险。移植物丢失,和死亡。需要更多的研究来确定血液学反应对每个MGRS相关病变结果的影响。
Data on kidney transplantation outcomes among patients with monoclonal gammopathy of renal significance (MGRS) are lacking.
Case series of patients with MGRS, some of whom received clone-directed therapies before kidney transplantation.
28 patients who underwent kidney transplantation from 1987 through 2016 after diagnosis with MGRS-associated lesions including light-chain deposition disease (LCDD), C3 glomerulopathy with monoclonal gammopathy (C3G-MG), and light-chain proximal tubulopathy (LCPT).
Of the 19 patients with LCDD, 10 were treated before kidney transplantation and 9 were treatment-naive. Among the treated patients with LCDD, 3 (30%) experienced histologic recurrence, 2 (20%) grafts failed, and 2 (20%) died during a median follow-up of 70 (range, 3-162) months after transplant. In the treatment-naive LCDD group, 8 (89%) had histologic recurrence, 6 (67%) grafts failed, and 4 (44%) patients died during a median follow-up of 60 (range, 35-117) months. Of the 5 patients who had a complete response before transplant, none died, and only 1 experienced graft failure, 162 months after transplant. Of 5 patients with C3G-MG, 3 were treatment-naive before transplant. Both patients who were treated before transplant had histologic recurrence, and 1 experienced graft failure and died. Among the 3 patients with treatment-naive C3G-MG, histologic recurrence occurred in all, and graft loss and death were observed in 2 and 1, respectively. In the LCPT group (n=4), histologic recurrence was observed in all 3 patients who did not receive clone-directed therapies before transplant, and 2 of these patients died, 1 with a functioning kidney. The 1 patient with LCPT who received therapy before transplant did not have histologic recurrence or graft loss and survived.
Small sample size, nonstandardized clinical management, retrospective design.
Recurrence is very common in all MGRS-associated lesions after kidney transplant. Achieving a complete hematologic response may reduce the risks of recurrence, graft loss, and death. More studies are needed to determine the effects of hematologic response on outcomes for each MGRS-associated lesion.