Department of Nephrology, Heraklion University Hospital, Crete, Greece.
Nephrol Dial Transplant. 2010 Jun;25(6):2020-3. doi: 10.1093/ndt/gfq023. Epub 2010 Feb 3.
Light-chain deposition disease (LCDD) is caused by an underlying clonal plasma cell dyscrasia in which monoclonal immunoglobulin light chains (LCs) are deposited in tissues, resulting in varying degrees of organ dysfunction. Autologous stem cell transplantation (ASCT) has been reported to stabilize renal function in patients with LCDD, but currently, no evidence of histopathologic resolution of LC deposition after ASCT exists. We present a patient, with severe renal dysfunction due to LCDD, who was treated with high-dose melphalan and ASCT that resulted in a significant and extended period of improved renal function. Four years after the initial improvement, the patient developed nephrotic range proteinuria, without any evidence of relapse of the plasma cell dyscrasia. At that time, a repeat renal biopsy showed complete resolution of LC depositions and development of extensive glomerulosclerosis, thus explaining proteinuria. To the best of our knowledge, this is the first report of a biopsy-proven resolution of renal LCDD following ASCT. A timely application of ASCT should be considered in LCDD to prevent deterioration of renal function in the long run.
轻链沉积病（LCDD）是由克隆性浆细胞异常引起的，其中单克隆免疫球蛋白轻链（LC）沉积在组织中，导致不同程度的器官功能障碍。自体干细胞移植（ASCT）已被报道可稳定 LCDD 患者的肾功能，但目前尚无 ASCT 后 LC 沉积的组织病理学缓解的证据。我们报告了 1 例因 LCDD 导致严重肾功能障碍的患者，该患者接受了大剂量马法兰和 ASCT 治疗，导致肾功能显著且长时间改善。初次改善 4 年后，患者出现肾病范围蛋白尿，无浆细胞异常复发的任何证据。当时，重复肾活检显示 LC 沉积完全缓解，并发展为广泛的肾小球硬化，从而解释了蛋白尿。据我们所知，这是首例 ASCT 后经活检证实的肾脏 LCDD 缓解的报告。在 LCDD 中应及时考虑应用 ASCT，以防止肾功能长期恶化。
