A successful treatment for chronic active Epstein-Barr virus disease with Nephrotic Syndrome.

IF 1 Q4 UROLOGY & NEPHROLOGY
CEN Case Reports Pub Date : 2024-06-01 Epub Date: 2023-09-05 DOI:10.1007/s13730-023-00815-5
Yasuhiro Inaba, Akinori Miyazono, Kenichi Imadome, Shinji Aratake, Yasuhiro Okamoto
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Abstract

Chronic active Epstein-Barr virus (CAEBV) disease is more likely to occur when a patient is on immunosuppressive therapy for any disease or is susceptible to infection, and the prognosis is poor without appropriate treatment, including hematopoietic stem cell transplantation (HSCT). In addition to HSCT, several other chemotherapy regimens have been reported, but all of them are difficult to maintain in remission. Without HSCT, survival rates have been reported to be 50% in 5 years and 25% in 15 years. This is a report of a 13-year-old boy who developed CAEBV disease during cyclosporine A (CyA) treatment for the steroid-dependent nephrotic syndrome (SDNS). Since SDNS precluded HSCT or chemotherapy, CyA was tapered off based on the belief that alleviating his immunosuppressed state would decrease the CAEBV disease. We decided to gradually reduce the CyA dose to activate T-cell immunity, while periodically monitoring the EBV viral load. Finally, we found an appropriate dose that could suppress both CAEBV disease and SDNS, and it lasted for more than 9 years. No case has been reported to date in which a patient developed CAEBV disease while receiving immunosuppressive drugs for the primary disease, and both diseases were controlled only by reducing the dose of immunosuppressive drugs. In this report, we show that dose reduction of immunosuppressive agents without chemotherapy or HSCT is an effective option for the treatment of CAEBV disease in patients receiving immunosuppressive agents.

成功治疗伴有肾病综合征的慢性活动性 Epstein-Barr 病毒病。
慢性活动性爱泼斯坦-巴氏病毒(CAEBV)病在患者因任何疾病接受免疫抑制治疗或易受感染时更易发生,如果不进行适当的治疗,包括造血干细胞移植(HSCT),预后很差。除造血干细胞移植外,还有其他几种化疗方案,但都难以维持缓解。据报道,如果不进行造血干细胞移植,5年存活率为50%,15年存活率为25%。本文报告了一名 13 岁男孩在环孢素 A(CyA)治疗类固醇依赖性肾病综合征(SDNS)期间罹患 CAEBV 的病例。由于 SDNS 排除了造血干细胞移植或化疗的可能性,我们认为减轻其免疫抑制状态会减少 CAEBV 疾病的发生,因此逐渐减少了 CyA 的用量。我们决定逐渐减少 CyA 剂量,以激活 T 细胞免疫,同时定期监测 EBV 病毒载量。最后,我们找到了一个既能抑制 CAEBV 病又能抑制 SDNS 的合适剂量,并且持续了 9 年多。迄今为止,还没有患者在接受免疫抑制剂治疗原发疾病的同时患上 CAEBV 病的病例报道,只有通过减少免疫抑制剂的剂量才能控制这两种疾病。在本报告中,我们证明了在不进行化疗或造血干细胞移植的情况下减少免疫抑制剂的剂量是治疗接受免疫抑制剂的患者 CAEBV 病的有效选择。
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来源期刊
CEN Case Reports
CEN Case Reports UROLOGY & NEPHROLOGY-
CiteScore
1.90
自引率
0.00%
发文量
80
期刊介绍: Clinical and Experimental Nephrology (CEN) Case Reports is a peer-reviewed online-only journal, officially published biannually by the Japanese Society of Nephrology (JSN).  The journal publishes original case reports in nephrology and related areas.  The purpose of CEN Case Reports is to provide clinicians and researchers with a forum in which to disseminate their personal experience to a wide readership and to review interesting cases encountered by colleagues all over the world, from whom contributions are welcomed.
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