总结 1.PNH发病机制是造血干细胞在一定条件下发生突变,产生GPI缺陷的克隆; 2.PNH是否溶血取决于红细胞GPI缺陷程度,外在诱因(睡眠等)会激活补体、加重红细胞破坏; 3.PNH伴随骨髓衰竭(AA,MDS)的分子机制尚不明确。 参考文献:[1]DACIE JV.Paroxysmal nocturnal haemoglobinuria.Proc R Soc Med.1963;56(7):587-596.[2]Dacie JV.Diagnosis and mechanism of hemolysis in chronic hemolytic anemia with nocturnal hemoglobinuria.Blood.1949;4(11):1183-1195.[3]Kinoshita T,Inoue N,Takeda J.Defective glycosyl phosphatidylinositol anchor synthesis and paroxysmal nocturnal hemoglobinuria.Adv Immunol.1995;60:57-103.[4]Parker CJ.Hemolysis in PNH.In:Young NS,Moss J,eds.Paroxysmal Nocturnal Hemoglobinuria and the Glycosylphosphatidylinositol-Linked Proteins.San Diego,CA:Academic Press;2000:49-100.[5]Parker CJ.Update on the diagnosis and management of paroxysmal nocturnal hemoglobinuria.Hematology Am Soc Hematol Educ Program.2016;2016(1):208-216.[6]PuJJ,Mukhina G,Wang H,Savage WJ,Brodsky RA.Natural history ofparoxysmal nocturnal hemoglobinuria clones in patients presenting asaplastic anemia.Eur J Haematol.2011 Jul;87(1):37-45.[7]WangSA,Pozdnyakova O,Jorgensen JL,et al..Detection ofparoxysmal nocturnal hemoglobinuria clones in patients withmyelodysplastic syndromes and related bone marrow diseases,withemphasis on diagnostic pitfalls and caveats.Haematologica.2009;94(1):29-37.[8]SugimoriC,Chuhjo T,Feng X,et al..Minor population of CD55-CD59-blood cells predicts response to immunosuppressive therapy andprognosis in patients with aplastic anemia.Blood.2006;107(4):1308-1314. 想要查看更多血液内容