摘要
目的总结造血干细胞移植后慢性移植物抗宿主病(cGVHD)相关的膜性肾病的诊疗体会。方法为1例急性淋巴细胞白血病患者施行HLA全相合的无关供者外周血造血干细胞移植,术后应用甲氨喋呤和他克莫司(FK506)预防GVHD。术后第19 d发生急性GVHD,经甲泼尼龙治疗逆转。分别于术后182 d、235 d停用FK506和泼尼松,5 d后患者出现cGVHD表现,肝功能异常,并伴肾病综合征的相关表现,病理诊断为膜性肾病Ⅱ期,遂给予FK506和泼尼松治疗,同时辅以利尿、降脂等措施。结果发生膜性肾病时,患者的尿蛋白++++,白细胞75个/μl,上皮细胞359.5个/μl,病理性管型+,管型计数为167个/μl,24 h尿蛋白定量为4.28 g;肾组织活检,无肾小球硬化,肾小球体积稍大,部分血管袢受压,开放欠佳,肾小球基底膜轻微增厚,外观呈僵硬感,系膜基质轻、中度增殖;间质区部分肾小管扩张,肿胀、变性,未见明显间质纤维化和炎症细胞浸润;M asson染色可见基底膜上皮侧嗜复红物沉积;免疫荧光检查,IgG+++,C3+++,IgA++,沿毛细血管袢呈颗粒状节段性分布,系膜区呈团块状分布;IgM、C1q、C4均阴性。电镜下可见肾小球基底膜上皮下沉积物,并有基膜增厚,毛细血管系膜基质轻度增生。经泼尼松和FK506治疗,2个月后尿蛋白转阴。结论造血干细胞移植后出现肾病综合征或蛋白尿,应考虑GVHD相关膜性肾病的可能,肾穿刺活检有助于诊断,糖皮质激素和FK506治疗有效。
Objective To sum up the clinical experience of the diagnosis and treatment of membranous glomerulopathy associated with chronic graft-versus-host disease （cGVHD） following allogeneic stem tell transplantation （alloSCT）. Methods One case of acute acute lymphoblastic leukemia received HLA complete matched alloSCT and subjected to methopterin and Tacrolimus （FK506） postoperatively for prevention of GVHD. Nineteen days after operation, the patient suffered from acute GVHD and recovered by using methylprednisolone. On the day of 182 and 235 after transplantation, FKS06 and prednisone were withdrawn. Five days later, cGVHD occurred, showing liver dysfunction and nephrotic syndrome. Membranous glomerulopathy （stage Ⅱ） was pathologically diagnosed and treated with FK506 and prednisone. Results In membranous glomerulopathy, the patient got albuminuria （ ＋ ＋ ＋ ＋ ）, leukocytes 75/μL, epicytes 359. 5/μL, pathological casts （ ＋ ）, 24-h urine protein 4. 28 g. Renal biopsy revealed that glomeruli were slightly enlarged, partial capillary compressed to be closed, glomerular basement membrane （GBM） was slightly thickened and mesangium slightly proliferate. Masson stain indicated the deposition of pink material along the glo- merular capillary walls. And IgG （ ＋ ＋ ＋ ）, C3 （ ＋ ＋ ＋ ） and IgA （ ＋ ＋ ） were detected at the GBM in a granular pattern by immunofluorescent stain. Electron microscopy showed numerous subepithelial deposits. After treatment with prednisone and FKS06 for 2 months, proteinuria was gradually decreased. Conclusions GVHD associated membranous glomerulopathy should be considered when the patient got nephrotic syndrome or proteinuria after alloSCT. Renal biopsy is beneficial to the diagnosis of GVHD. Glucocorticoid and FK506 is effective in the treatment of GVHD.
出处
《中华器官移植杂志》
CAS
CSCD
北大核心
2006年第3期177-179,共3页
Chinese Journal of Organ Transplantation
关键词
造血干细胞移植
移植物抗宿主病
肾小球肾炎
膜性
Hematopoietic stem cell transplantation
Graft vs host disease
Glomerulonephritis,membranous
