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在肾移植或不进行干细胞移植的情况下,轻链淀粉样变性(AL)患者的长期预后。

Long-term outcomes of patients with light chain amyloidosis (AL) after renal transplantation with or without stem cell transplantation.

机构信息

Division of Nephrology and Hypertension, Mayo Clinic Rochester, Rochester, MN, USA.

出版信息

Nephrol Dial Transplant. 2011 Jun;26(6):2032-6. doi: 10.1093/ndt/gfr067. Epub 2011 May 4.

Abstract

BACKGROUND

Recent advances in the treatment of immunoglobulin light chain amyloidosis (AL) have dramatically improved survival. Kidney transplantation (KTx) has become more common but the long-term outcomes remain unknown and it is the objective of this study.

METHODS

Nineteen patients with AL underwent living (n = 18) or deceased (n = 1) KTx at our institution from 1999 to 2008 (median age 57 years, six women). The primary end points were patient and kidney allograft survival and recurrence of AL in the allograft. The secondary end point was kidney transplant rejection. Outcome data were stratified according to three treatment modalities: renal transplantation followed by autologous stem cell transplantation (ASCT) (Group 1, n = 8), ASCT followed by renal transplantation (Group 2, n = 6) and renal transplantation after complete remission achieved with nonmyeloablative therapy (Group 3, n = 5).

RESULTS

The median follow-up was 41.4 months. At the time of study, 79% were still alive. Median graft survival did not differ from median overall survival. There was no difference in survival rates between the treatment groups. Five patients had a cellular rejection. Two of the three patients with a rejection in Group 1 died but neither patient with rejection in Groups 2 and 3. Recurrent amyloidosis was diagnosed by biopsy in one patient in Group 2 (preceding ASCT) and in another patient in Group 3.

CONCLUSIONS

KTx can be successfully performed in AL patients in complete hematologic response and meet the usual KTx selection criteria. Outcomes appear similar whether hematologic response was achieved with ASCT or by nonmyeloablative therapy.

摘要

背景

免疫球蛋白轻链淀粉样变性(AL)治疗的最新进展显著改善了患者的生存率。肾移植(KTx)变得更为常见,但长期结果仍不清楚,这也是本研究的目的。

方法

1999 年至 2008 年期间,18 名患者接受了活体(n=18)或尸体(n=1)肾移植,我们机构的患者患有 AL。主要终点是患者和肾移植的存活率以及移植肾中 AL 的复发。次要终点是肾移植排斥。根据三种治疗方式对结果数据进行分层:肾移植后自体干细胞移植(ASCT)(第 1 组,n=8)、ASCT 后肾移植(第 2 组,n=6)和非清髓性治疗达到完全缓解后肾移植(第 3 组,n=5)。

结果

中位随访时间为 41.4 个月。研究时,79%的患者仍然存活。移植肾中位存活率与总中位存活率无差异。各组之间的生存率没有差异。5 例患者发生细胞性排斥。第 1 组中 2 例发生排斥反应的患者死亡,但第 2 组和第 3 组中无发生排斥反应的患者死亡。第 2 组中有 1 例患者和第 3 组中有 1 例患者因活检诊断为复发性淀粉样变性,此前该患者接受了 ASCT。

结论

在完全血液学缓解的情况下,AL 患者可以成功进行 KTx,并符合通常的 KTx 选择标准。无论血液学反应是通过 ASCT 还是非清髓性治疗实现,结果似乎相似。

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