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目的 探讨异基因造血干细胞移植 (HSCT)后表现在肾脏的慢性移植物抗宿主病(GVHD)的临床及病理特点。方法 通过 1例异基因HSCT患者GVHD的临床及病理资料并结合文献复习 ,对其临床表现和组织形态学的多样性以及与治疗的关系进行综合分析。结果 HSCT后出现肾小球性蛋白尿者虽未经统计 ,但本院实际已见数例 ,仅此例进行了肾穿刺活检。临床表现为肾病综合征 (NS) ,同时有口角炎、皮肤鱼鳞状改变并存。组织学呈非典型膜性肾病 :肾小球毛细血管襻不规则轻至重度增厚 ;免疫病理显示IgG沿毛细血管襻呈颗粒状沉积 ;电子显微镜下可见肾小球毛细血管襻上皮下及系膜区中等量电子致密物沉积。结论 HSCT后发生的NS可能是一自身免疫性疾病 ,是移植物对机体产生的一种排斥反应 ,随HSCT技术的逐渐开展 ,该病发生率渐增多。肾穿刺活检对该病的诊断有重要帮助 ;加强免疫抑制治疗可减少该病发生 ,骁悉在该病的治疗中有明确疗效
Objective To investigate the clinical and pathological features of chronic graft-versus-host disease (GVHD) in kidney after allogeneic hematopoietic stem cell transplantation (HSCT). Methods The clinical and pathological data of GVHD in one patient with allogeneic HSCT were reviewed, and the clinical manifestations, the diversity of histomorphology and the relationship with the treatment were analyzed comprehensively. Results Although glomerular proteinuria occurred after HSCT, although there were no statistics, there were actually several cases in our hospital. Only the renal biopsy was performed in this case. Clinical manifestations of nephrotic syndrome (NS), while angular cheilitis, squamous changes in skin coexist. Histopathology showed atypical membranous nephropathy: glomerular capillary loop irregular light to severe thickening; immunopathological IgG showed granular deposition along the capillaries; electron microscopy showed glomerular capillary loop subcutaneous and Department Membrane area in the amount of electron density deposition. Conclusion The NS that occurs after HSCT may be an autoimmune disease and is an allograft rejection reaction to the body. With the gradual development of HSCT, the incidence of this disease is increasing. Kidney biopsy of the diagnosis of the disease have important help; strengthening immunosuppressive therapy can reduce the occurrence of the disease, the definitive treatment of the disease has a clear effect