Haseyama K, Watanabe J, Oda T, Katoh S, Suzuki N, Kudoh T, Chiba S
Department of Pediatrics, Sapporo Medical University School of Medicine.
Rinsho Ketsueki. 1996 Dec;37(12):1383-8.
A 13-yr-old boy was diagnosed as T cell lymphoma. After the second remission, he underwent BMT from an HLA-identical, MLC negative sibling donor. After BMT, he developed grade II acute GVHD. GVHD was improved by pulsed steroid therapy using prednisolone. About 12 months after BMT, he developed bronchiolitis obliterans, sicca syndrome, and leukoderma, which were related to chronic GVHD. Pulsed steroid therapy was carried out twice, and his condition improved. Twenty-seven months after BMT, he developed nephrotic syndrome. A renal biopsy was performed, and the diagnosis was histologically membranous nephropathy and focal glomerular sclerosis. The response to steroids was not satisfactory. After 5 weeks, dipyridamole was added, but proteinuria persisted. Proteinuria disappeared 8 weeks after the addition of cyclosporine. The second biopsy after 5 months of treatment revealed an improvement in the renal lesions. The patient showed a low T4 to T8 ratio of T-lymphocytes at the onset of nephrotic syndrome. However after treatment with cyclosporine, the ratio gradually increased. These findings suggested the nephrotic syndrome in this patient was related to renal involvement in the course of chronic GVHD.
一名13岁男孩被诊断为T细胞淋巴瘤。第二次缓解后，他接受了来自HLA相同、混合淋巴细胞培养阴性的同胞供体的骨髓移植。骨髓移植后，他发生了II级急性移植物抗宿主病（GVHD）。通过使用泼尼松龙的脉冲类固醇疗法，GVHD得到改善。骨髓移植后约12个月，他出现了闭塞性细支气管炎、干燥综合征和白斑，这些都与慢性GVHD有关。进行了两次脉冲类固醇疗法，他的病情有所改善。骨髓移植27个月后，他出现了肾病综合征。进行了肾活检，组织学诊断为膜性肾病和局灶性肾小球硬化。对类固醇的反应并不令人满意。5周后，加用双嘧达莫，但蛋白尿持续存在。加用环孢素8周后蛋白尿消失。治疗5个月后的第二次活检显示肾脏病变有所改善。该患者在肾病综合征发病时T淋巴细胞的T4与T8比值较低。然而，在用环孢素治疗后，该比值逐渐升高。这些发现提示该患者的肾病综合征与慢性GVHD病程中的肾脏受累有关。
