【摘要】 目的:探讨移植相关性血栓性微血管病(transplantation-associated thrombotic microangiopathy,TA-TMA)肾损伤的临床和病理特点及其病因和发病机制。方法:以造血干细胞移植和同种异体肾移植为例,将其肾穿刺活检标本分别进行免疫荧光、光学显微镜和透射电子显微镜的病理学观察。结果:患者在接受移植后的不同时间内,出现高血压、大量蛋白尿、血管内溶血性贫血及肾功能减退;免疫荧光检查显示,各种免疫球蛋白及补体均阴性;光学显微镜检查显示,肾小球和肾小动脉内皮细胞增生、肿胀,基底膜不规则增厚,可有微血栓形成;透射电子显微镜检查显示,除内皮细胞增生、肿胀外,基底膜内疏松层增厚,未见电子致密物。结论:TA-TMA是器官或组织移植后较少见的并发症。TA-TMA常累及肾脏,出现大量蛋白尿、贫血及肾功能减退。应与常见的排异反应或其他肾疾病鉴别,肾穿刺活检是确诊的主要方法。TA-TMA的发生与抗排异反应的免疫抑制剂的应用和病毒感染并导致内皮细胞损伤有关。更多还原

【Abstract】 Objective:To study clinicopathological features and mechanism of renal injury in transplantation-associated thrombotic microangiopathy (TA-TMA). Methods:The renal biopsies obtained from patients after hematopoietic cell transplantation or kidney transplantation were observed by immunofluorescence, light microscopy and electron microscopy (EM). Results:The patients presented hypertension, massive proteinuria, intravascular haemolysis and renal insufficiency some time after transplantation. Various immunoglobulins and complements were negative in immunofluorescent staining. Light microscopy showed endothelial cell proliferation and swelling of glomerular capillary and small arteries, irregular thickened glomerular basement membrane and microthrombosis. EM revealed endothelial cell proliferation and swelling with thickened lamina rara interna of glomerular basement membrane. Conclusion:TA-TMA is a rare complication after organ or tissue transplantation. Kidney is often involved in TA-TMA with massive proteinuria, anemia and renal failure. Differential diagnosis should be made between TA-TMA and rejection or other renal diseases. Renal biopsy is a major method for accurate diagnosis. TA-TMA correlates to endothelial cell injury caused by viral infection and/or immunosuppressive drugs for anti-rejection.更多还原