Miyamoto Satoshi, Kimura Shunsuke, Hosoya Yosuke, Hasegawa Daisuke, Ishida Hisashi, Daida Atsuro, Matsui Toshihiro, Yoshimoto Yuri, Hirabayashi Shinsuke, Fujimaru Takuya, Kumamoto Tadashi, Mori Shin-Ichiro, Suzuki Koyu, Manabe Atsushi
Department of Pediatrics, St. Luke's International Hospital.
Department of Pediatrics, Tokyo Medical and Dental University.
Rinsho Ketsueki. 2018;59(4):389-394. doi: 10.11406/rinketsu.59.389.
An eight-year-old girl with myelodysplastic syndrome (refractory cytopenia) received a bone marrow transplant (BMT) from an unrelated donor because of immunosuppressive therapy failure. Following administration of foscarnet for cytomegalovirus reactivation at day40 post-BMT, serum creatinine increased, and proteinuria, hematuria, and hypertension gradually exacerbated and became prolonged. However, neither schistocytosis nor other organ damage was evident. At six months post-BMT, renal biopsy revealed diffuse glomerular damage with glomerular lobulation, a double contour of the glomerular basement membrane, erythrocyte congestion and thrombi in the glomerular endocapillaries, and mesangiolysis, confirming the diagnosis of transplantation-associated thrombotic microangiopathy (TA-TMA). We initiated strict controls regarding fluid balance, salt intake, and blood pressure. The patient's renal function improved 10 months post-BMT. TA-TMA often presents as non-specific symptoms, making diagnosis difficult. In cases of post-transplant renal damage, TA-TMA should be differentiated regardless of whether specific symptoms such as hemolytic anemia and other organ failure are evident, and a renal biopsy should, therefore, be considered.
一名患有骨髓增生异常综合征（难治性血细胞减少症）的8岁女孩因免疫抑制治疗失败，接受了来自非亲属供体的骨髓移植（BMT）。在BMT后第40天因巨细胞病毒再激活给予膦甲酸钠治疗后，血清肌酐升高，蛋白尿、血尿和高血压逐渐加重且持续时间延长。然而，未见裂体细胞增多或其他器官损害。BMT后6个月，肾活检显示弥漫性肾小球损害，伴有肾小球分叶、肾小球基底膜双轨征、肾小球毛细血管内红细胞淤滞和血栓形成以及系膜溶解，确诊为移植相关血栓性微血管病（TA-TMA）。我们开始对液体平衡、盐摄入和血压进行严格控制。患者的肾功能在BMT后10个月有所改善。TA-TMA常表现为非特异性症状，诊断困难。在移植后肾损害的病例中，无论是否有溶血性贫血和其他器官衰竭等特异性症状，均应鉴别TA-TMA，因此应考虑进行肾活检。
