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长期缓解后肾移植中轻链沉积病的复发。

Light Chain Deposition Disease Recurrence in Renal Allograft after Long-Term Remission.

机构信息

Kidney Center, Japanese Red Cross Aichi Medical Center Nagoya Daini Hospital, Nagoya, Japan.

出版信息

Nephron. 2023;147 Suppl 1:96-100. doi: 10.1159/000529776. Epub 2023 Feb 21.

Abstract

Light chain deposition disease (LCDD) is a rare manifestation of monoclonal gammopathy, which can lead to renal failure. We previously reported a detailed recurrence process in a case of LCDD after renal transplantation. To the best of our knowledge, no report has described the long-term clinical course and renal pathology findings of recurrent LCDD in patients after renal transplantation. In this case report, we describe the long-term clinical presentation and changes in renal pathology of the same patient after early LCDD relapse in a renal allograft. A 54-year-old woman with recurrent immunoglobulin A λ-type LCDD in an allograft was admitted 1 year post-transplant for bortezomib and dexamethasone therapy. At 2 years post-transplantation, a graft biopsy performed after complete remission was achieved, showing some glomeruli with residual nodular lesions similar to the pre-treatment renal biopsy findings. However, the enlarged subendothelial space disappeared. She remained in complete remission serologically for 6 years. Subsequently, the ratio of serum κ/λ-free light chains decreased gradually. She underwent a transplant biopsy approximately 12 years after renal transplantation due to increased proteinuria and decreased renal function. Compared with the previous graft biopsy, almost all glomeruli showed advanced nodule formation and subendothelial expansion. Because the LCDD case relapsed after long-term remission following renal transplantation, protocol biopsy monitoring might be necessary.

摘要

轻链沉积病 (LCDD) 是单克隆丙种球蛋白病的一种罕见表现,可导致肾衰竭。我们之前报道了一例肾移植后 LCDD 的详细复发过程。据我们所知,尚无报告描述肾移植后 LCDD 复发患者的长期临床病程和肾脏病理发现。在本病例报告中,我们描述了同一位患者在肾移植后早期 LCDD 复发后的长期临床表现和肾脏病理变化。一名 54 岁女性,在移植后 1 年因复发性免疫球蛋白 A λ 型 LCDD 入院接受硼替佐米和地塞米松治疗。移植后 2 年,在完全缓解后进行的移植肾活检显示,一些肾小球仍存在与治疗前肾活检相似的残留结节病变。然而,扩大的内皮下空间已消失。她在血清学上持续缓解 6 年。随后,血清 κ/λ 游离轻链的比值逐渐降低。她在肾移植后约 12 年因蛋白尿增加和肾功能下降而接受移植肾活检。与之前的移植肾活检相比,几乎所有肾小球均显示出晚期结节形成和内皮下扩张。由于 LCDD 病例在肾移植后长期缓解后复发,可能需要进行方案活检监测。

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