Matsuura Tomokazu, Hiramoto Nobuhiro, Iwasa Takeshi, Shoji Masaaki, Ohashi Ken
Department of Internal Medicine, National Cancer Center Hospital, Tokyo, Japan.
Nihon Jinzo Gakkai Shi. 2013;55(7):1327-34.
A 64-year-old man with treatment-related myelodysplastic syndrome (MDS) underwent non-myeloablative allogeneic peripheral blood stem cell transplantation from a fully HLA-matched sibling. Seven months after transplantation, he suffered from nephrotic syndrome (proteinuria 16.67 g/day) around two weeks atter tapering tacrolimus (TAC) for the prophylaxis of graft-versus-host disease(GVHD). A renal biopsy revealed membranous nephropathy (Stage I ). Treatment with prednisolone (PSL), starting with 50 mg daily, resulted in incomplete remission type I. Although remission was maintained for 7 months, nephrotic syndrome recurred (proteinuria 7.81 g/day)after tapering PSL(5 mg/day) (18 months after transplantation). His PSL dose was increased again to 50 mg daily, and proteinuria improved again. Two weeks after discontinuation of TAC as it was suspected of worsening his renal function, proteinuria increased again to 6.37 g/day (21.5 months after transplantation). After administration of cyclosporin A (CsA) (30 mg/day) instead of TAC, proteinuria re-improved and complete remission of nephrotic syndrome was achieved. In this case, nephrotic syndrome worsened twice just after tapering or discontinuing immunosuppressive medication, and reinforcement of immunosuppression was effective in improving proteinuria. As hematopoietic cell transplantation (HCT) is an increasingly common treatment worldwide, the opportunities to see patients with nephrotic syndrome after HCT are also increasing. Our case serves as a reference to manage the recurrence of nephrotic syndrome after HCT.
一名64岁患有治疗相关骨髓增生异常综合征（MDS）的男性患者，接受了来自完全人类白细胞抗原（HLA）匹配同胞的非清髓性异基因外周血干细胞移植。移植后7个月，在逐渐减少用于预防移植物抗宿主病（GVHD）的他克莫司（TAC）约两周后，他患上了肾病综合征（蛋白尿16.67克/天）。肾活检显示为膜性肾病（I期）。从每日50毫克开始使用泼尼松龙（PSL）治疗，导致不完全缓解I型。尽管缓解维持了7个月，但在逐渐减少PSL（5毫克/天）后（移植后18个月）肾病综合征复发（蛋白尿7.81克/天）。他的PSL剂量再次增加至每日50毫克，蛋白尿再次改善。由于怀疑TAC会恶化他的肾功能而停用TAC两周后，蛋白尿再次增加至6.37克/天（移植后21.5个月）。在改用环孢素A（CsA）（30毫克/天）替代TAC后，蛋白尿再次改善，肾病综合征实现了完全缓解。在该病例中，肾病综合征在逐渐减少或停用免疫抑制药物后仅两次恶化，加强免疫抑制对改善蛋白尿有效。由于造血细胞移植（HCT）在全球范围内是一种越来越常见的治疗方法，HCT后出现肾病综合征患者的机会也在增加。我们的病例可为管理HCT后肾病综合征的复发提供参考。
