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A long-term remission of renal amyloidosis with nephrotic syndrome after autologous peripheral blood stem-cell transplantation

机译:自体外周血造血干细胞移植后肾病综合征长期缓解与肾病综合征

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摘要

Renal amyloidosis is typically characterized by nephrotic syndrome, often with massive proteinuria and refractory peripheral edema. We report the case of a patient with renal amyloidosis associated with nephrotic syndrome who maintained remission for 6 years after undergoing high-dose chemotherapy followed by autologous peripheral blood stem-cell transplantation (auto-PBSCT). The patient was a man aged in his 50s who had developed nephrotic syndrome. Bone marrow aspiration and kidney biopsy determined that the cause of the nephrotic syndrome was renal amyloidosis due to multiple myeloma, and the patient was admitted to our department in July 2003. After one course of chemotherapy, auto-PBSCT was performed in March 2004. Following transplantation, serum M-protein was no longer detectable from March 2005, and the patient achieved complete hematological remission. Subsequently, proteinuria decreased, serum albumin levels normalized, and nephrotic syndrome improved. As of 6 years after transplantation, in March 2010, the patient remained in remission, meaning that auto-PBSCT proved extremely effective as a treatment for renal amyloidosis in this case.
机译:肾淀粉样变性病通常以肾病综合征为特征,通常伴有大量蛋白尿和难治性外周水肿。我们报道了一例伴有肾病综合征的肾淀粉样变性病患者,该患者在接受大剂量化学疗法后自体外周血干细胞移植(auto-PBSCT)保持了6年的缓解。该患者是一名50多岁的患有肾病综合症的人。骨髓穿刺和肾脏活检确定肾病综合征的病因是多发性骨髓瘤引起的肾脏淀粉样变性,该患者于2003年7月入院。经过一个疗程的化疗,于2004年3月进行了自动PBSCT。移植后,从2005年3月开始不再检测到血清M蛋白,患者完全血液学缓解。随后,蛋白尿减少,血清白蛋白水平恢复正常,肾病综合征改善。移植后6年,2010年3月,患者仍处于缓解状态,这意味着auto-PBSCT在这种情况下被证明对治疗肾淀粉样变性病非常有效。

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