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硼替佐米成功逆转肾移植中轻链沉积病的早期复发:一例报告

Bortezomib successfully reverses early recurrence of light-chain deposition disease in a renal allograft: a case report.

作者信息

Kaposztas Z, Kahan B D, Katz S M, Van Buren C T, Cherem L

机构信息

Department of Surgery, University of Texas Medical School at Houston, Houston, Texas 77030, USA.

出版信息

Transplant Proc. 2009 Dec;41(10):4407-10. doi: 10.1016/j.transproceed.2009.10.005.

Abstract

Light-Chain Deposition Disease (LCDD) frequently recurs after renal transplantation, displaying a pernicious course. Herein we have described a 39-year-old Caucasian man with a history of immunoglobulin G-kappa multiple myeloma who failed two chemotherapy regimens, but ultimately responded to the combination of thalidomide, bortezomib, and dexamethasone followed by high-dose melphalan and autologous stem cell transplantation 3 years prior to transplantation, during which time he showed no evidence of persistent or recurrent disease. At 3 days following spousal living related renal transplantation, he displayed a rapid deterioration of renal function requiring dialysis therapy. This episode failed to respond to empiric antirejection therapy including anti-thymocyte globulin, plasmapheresis, and anti-CD20 monoclonal antibody. Increasing evidence suggested recurrence of LCDD, including positive immunofluorescence staining of basement membranes and vessels for kappa light chains as well as free kappa light chains in his urine and serum. Following suspension of sirolimus, he was initiated on and responded to bortezomib (1.3 mg/m(2)) with discontinuation of dialysis within 3 weeks and progressively improving renal function. His maintenance therapy, in addition to six 2-week-long cycles of bortezomib separated by 1-week rest periods, includes cyclosporine (50 mg twice daily), prednisone (10 mg daily), and curcumin (9 g daily). In sum, bortezomib rescue therapy salvaged a spousal renal transplant afflicted with recurrent LCDD.

摘要

轻链沉积病(LCDD)在肾移植后常复发,病程凶险。在此,我们描述了一名39岁的白人男性,有免疫球蛋白G-κ多发性骨髓瘤病史,两种化疗方案均失败,但最终对沙利度胺、硼替佐米和地塞米松联合治疗有反应,随后在移植前3年接受了大剂量美法仑和自体干细胞移植,在此期间他没有持续性或复发性疾病的证据。在配偶活体亲属肾移植术后3天,他的肾功能迅速恶化,需要透析治疗。这一情况对包括抗胸腺细胞球蛋白、血浆置换和抗CD20单克隆抗体在内的经验性抗排斥治疗无反应。越来越多的证据表明LCDD复发,包括基底膜和血管κ轻链免疫荧光染色阳性以及他的尿液和血清中游离κ轻链。停用西罗莫司后,他开始使用硼替佐米(1.3 mg/m²)治疗并产生反应,3周内停止透析,肾功能逐渐改善。他的维持治疗除了每2周进行6个周期、间隔1周休息的硼替佐米治疗外,还包括环孢素(每日两次,每次50 mg)、泼尼松(每日10 mg)和姜黄素(每日9 g)。总之,硼替佐米挽救治疗使一例受复发性LCDD折磨的配偶肾移植得以挽救。

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