异基因外周血造血干细胞移植后膜性肾病临床和病理表现

Membranous nephropathy after allogeneic peripheral blood hematopoietic stem cell transplantation --Clinicopathologic manifestations of a rare chronic GVHD

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 。

Objective To investigate the clinicopathologic characteristics of renal chronic graft versus host disease (GVHD) after allogeneic peripheral blood hematopoietic transplantation(HSCT). Methods Clinical and pathological data from a patient with post BMT GVHD were analysed with a related references review. Result The patient developed nephrotic syndrome (NS)8 months after allogeneic HSCT.Renal biopsy revealed irregular thickening of glomerular basement membrane with granular deposits of IgG along it. Electronic microscopy showed electron dense deposits with increased mesangial matrix and cells in subepithelial and mesangial area. Conclusion NS occurred after HSCT might be an autoimmune glomerulonephritis. Immune suppressor could reduce the incidence of NS in the HSCT patients. Cell cept had a good efficacy in the treatment and renal biopsy played an important role in the diagnosis and prognosis of renal cGVHD.