Eudcation Center, National Cheng Kung University Hospital, Tainan, Taiwan.
Division of Pediatric Hematology, Department of Pediatrics, College of Medicine, National Cheng Kung University Hospital, Tainan, Taiwan.
BMC Nephrol. 2020 Nov 17;21(1):490. doi: 10.1186/s12882-020-02136-6.
In patients with frequently relapsing nephrotic syndrome, immunosuppressive therapy such as cyclosporine are often required to maintain remission. Cyclosporine has been noted to have tumorgenesis effects. In this case report, we present a child with relapsing nephrotic syndrom developed a rhabdomyosarcoma on her tongue after adout 4 years of continual immunosuppressive therapy.
A 2-year-old female child had nephrotic syndrome (urine protein-creatinine ratio 749.1 mg/mg; blood urea nitrogen 11 mg/dL; serum creatinine 0.3 mg/dL; and serum albumin 1.8 g/dL.) Proteinuria resolved on treatment with daily prednisolone for 4 weeks at the dose of 45 mg (2.5 mg/kg/day) but recurred with taper from 25 mg/day to 10 mg/day. At least five more episodes of relapse occurred within about a 3-year period. After the third relapse, she was treated with prednisolone and cyclosporine (at initial dose of 50 mg/day [1.7 mg/kg/day]) for immunosuppression. About 4 years after the diagnosis of nephrotic syndrome had been made, an embryonal rhabdomyosarcoma developed on her tongue. The cancer was treated with TPOG-RMS-LR protocol, with vincristine, actinomycin, and cyclophosphamide. Magnetic resonance imaging scan, performed about 3 years after the start of TPOG-RMS-LR therapy, revealed complete remission of the cancer.
Although treatment with cyclosporine cannot be conclusively implicated as the cause the rhabdomyosarcoma in this patient, the association should prompt consideration of its use in the treatment of frequently relapsing nephrotic syndrome in children.
在频繁复发的肾病综合征患者中,通常需要免疫抑制疗法,如环孢素,以维持缓解。环孢素有致癌作用。在本病例报告中,我们介绍了一名儿童,在持续免疫抑制治疗约 4 年后,其舌部发生了横纹肌肉瘤,此前曾反复发作肾病综合征。
一名 2 岁女性患儿患有肾病综合征(尿蛋白-肌酐比值 749.1mg/mg;血尿素氮 11mg/dL;血清肌酐 0.3mg/dL;血清白蛋白 1.8g/dL)。给予泼尼松龙治疗 4 周(剂量为 45mg,即 2.5mg/kg/日)后蛋白尿得到缓解,但从 25mg/日逐渐减量至 10mg/日时病情复发。在大约 3 年的时间内,至少又发生了 5 次复发。第三次复发后,她开始接受泼尼松龙和环孢素(初始剂量为 50mg/日[1.7mg/kg/日])治疗以抑制免疫。在肾病综合征确诊约 4 年后,她的舌部发生了胚胎性横纹肌肉瘤。该癌症采用 TPOG-RMS-LR 方案进行治疗,使用了长春新碱、放线菌素和环磷酰胺。在开始 TPOG-RMS-LR 治疗约 3 年后进行的磁共振成像扫描显示癌症完全缓解。
虽然不能确定环孢素治疗是导致该患者横纹肌肉瘤的原因,但这种关联应促使人们考虑在治疗儿童频繁复发的肾病综合征时使用环孢素。