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目的:探讨移植后并发毛细血管渗漏综合症(capillary leak syndrome,CLS)和可逆性后部白质脑病综合征(Posterior reversibleencephalopathy syndrome,PRES)的相关发病因素及治疗措施,并探索其与阵发性睡眠性血红蛋白尿(Paroxysmal NocturnalHemoglobinuria,PNH)的相关性及其防治措施。方法:本文报道一例PNH患者行非血缘供者外周血造血干细胞移植(allo-HSCT)后先后并发CLS和PRES患者的病历资料,并进行相关的文献复习。结果:患者确诊PNH,于我院行allo-HSCT后先后并发CLS和PRES,经积极治疗后好转。复习相关文献发现CLS和PRES均与内皮细胞损害相关,而PNH同样存在内皮损伤的基础病变,去纤苷、抗凝血酶Ⅲ和Eculizumab在类似疾病中有内皮细胞保护作用。结论:HSCT后先后并发CLS和PRES的病例临床非常少见,两者均与内皮细胞损伤相关。PNH本身存在内皮细胞损伤,是移植后易并发内皮细胞损伤相关并发症的原因之一。PNH行allo-HSCT预处理方案应避免选用高内皮细胞毒性方案,去纤苷、抗凝血酶Ⅲ和Eculizumab是行HSCT值得考虑内皮细胞保护剂。
OBJECTIVE: To investigate the related pathogenesis and treatment of capillary leak syndrome (CLS) and reversible posterior reversible encephalopathy syndrome (PRES) after transplantation, and to explore its relationship with paroxysmal Relativity and Prevention and Cure Measures of Paroxysmal Nocturnal Hemoglobinuria (PNH). Methods: This article reports a case of PNH patients with non-blood donor peripheral blood hematopoietic stem cell transplantation (allo-HSCT) followed by CLS and PRES patients with medical records, and relevant literature review. Results: Patients diagnosed PNH, in our hospital after allo-HSCT has concurrent CLS and PRES, improved after active treatment. Review of relevant literature found that CLS and PRES are associated with endothelial cell damage, and PNH also exists the basic lesion of endothelial lesions, defibrotide, antithrombin Ⅲ and Eculizumab have endothelial cell protection in similar diseases. CONCLUSION: CLS and PRES cases are rare after HSCT, both of which are associated with endothelial cell injury. PNH itself is endothelial cell injury, is one of the causes of complications associated with endothelial cell injury after transplantation. PNH line allo-HSCT pretreatment program should avoid the use of high endothelial cell toxicity programs, defibrotide, antithrombin Ⅲ and Eculizumab line HSCT worth considering endothelial protectants.