Observations on red cell membrane of paroxysmal nocturnal hemoglobinuria
Zhang, Z.N.; Pan, H.Z.; Huang, W.X.; Zhou, F.L.; Feng, L.M.; Li, C.H.; Fang, Z.Y.; Xiong, F.; Hong, T.; Zhang, A.
Chinese Medical Journal 94(4): 207-212
1981
ISSN/ISBN: 0366-6999 PMID: 6790235 Document Number: 178104
Paroxysmal nocturnal hemoglobinuria (PNH) is a common hemolytic disease in north China and may be a good field for RBC membrane study. Cobra venom factor test was simplified and used as a routine PNH diagnostic procedure. Scanning electron microscope observations showed no unique PNH characteristics, but could be utilized as a supplementary means for diagnosing hemolytic anemia. Studies of the hemolysis mechanism revealed that the C3 receptors were not increased on PNH RBC surfaces and that the reason for raised complement susceptibility should be sought elsewhere. RBC membrane protein by sodium dodecyl sulfate-polyacrylamide gel electrophoresis showed a band VI increase in some PNH cases, while the acetylcholine-esterase activity of red blood cell membrane and SH radical content were lowered in some. RBC of some PNH patients hemolyzed less after incubation with neuramidinase. The diversity of results among different patients probably reflects PNH complexity and heterogenicity of RBC membrane defects and disparity of clinical manifestations. PNH seems to be a syndrome rather than a single disease.