PNH行allo-HSCT后并发毛细血管渗漏综合征和可逆性后部白质脑病综合征一例并文献复习  

Capillary Leak Syndrome and Posterior Reversible Encephalopathy Syndrome after allo-HSCT in PNH: a Case Report with Literature Review

作　　者：臧学峰[1,2,3,4] 江岷[2,3,4] 楼晓[2,3,4] 李渤涛[2,3,4] 庞一琳[1,2,3,4] 刘婷婷[2,3,4] 李倩[2,3,4] 胡亮钉[2,3,4] 陈虎[2,3,4] 

机构地区：[1]解放军军医进修学院,北京100853 [2]全军造血干细胞研究所,北京100071 [3]军事医学科学院附属医院造血干细胞移植科,北京100071 [4]军事医学科学院细胞与基因治疗中心,北京100071

出　　处：《现代生物医学进展》2013年第8期1471-1474,共4页Progress in Modern Biomedicine

基　　金：国家"863"重大专项课题(2011AA020114);首都临床特色应用研究(SQ2010AA0201008009)

摘　　要：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 study whether the occurrence of capillary leak syndrome （CLS） and posterior reversible encephalopathy syndrome （PRES） after hematopoietic stem cell transplantation （HSCT） had a relationship with Paroxysmal Nocturnal Hemoglobinuria （PNH）, and their preventative measures. Methods： One case of PNH after aUo-HSCT complicated with CLS and PRES was reported with its related literature being reviewed. Results： The patient was definitively diagnosed as PNH. After treatment of allo-HSCT, she was complicated by CLS and PRES sequently. Related literatures were reviewd, it was found that CLS and PRES were all related to the damage of endothelial cells. Besides, PNH could lead to endothelial cell damage. Defibrotide, antithrombin III and eculizumab were all used in similar disease as an endothelial cell protector role. Conclusion： CLS and PRES successively happened after HSCT were very rare, both of which were related to endothelial cell injury. PNH may lead to endothelial cell injury, which was one reason for inclined endothelial cell damage-related complications after transplantation. High endothelial cell toxicity conditioning regiment should be avoid to PNHs who were undergoing a allo-HSCT treatment. Defibrotide, antithrombin III and eculizumab were potential endothelial cell protective agents for HSCT.

关 键 词：异基因造血干细胞移植 毛细血管渗漏综合症 可逆性后部白质脑病综合征 内皮细胞损伤 内皮细胞保护剂 

分 类 号：R557.3[医药卫生—血液循环系统疾病]