Bogusławska D M, Heger E, Chorzalska A, Nierzwicka M, Hołojda J, Swiderska A, Straburzyńska A, Paździor G, Langner M, Sikorski A F
Institute of Biotechnology and Environmental Sciences, University of Zielona Góra, ul. Monte Cassino 21B, 65-561 Zielona Góra, Poland.
Ann Hematol. 2004 Jan;83(1):28-33. doi: 10.1007/s00277-003-0739-5. Epub 2003 Sep 27.
Red blood cells of 17 patients out of seven families diagnosed with HS from the southwest of Poland were studied. In six families a deficiency of ankyrin was detected, and in one family a band 3 (anion-exchanger protein) deficiency was detected. Patients from six families with the ankyrin deficiency had a 19-51% decrease in ankyrin 2.1, while the family with the band 3 deficiency showed a 33% decrease in this protein content. All changes were statistically significant, as analysed by the Student t test (P<0.05). Analysis of haemolysis kinetics gives a reliable indication of altered osmotic properties of the spherocytic cells.
对来自波兰西南部被诊断为遗传性球形红细胞增多症(HS)的7个家族的17名患者的红细胞进行了研究。在6个家族中检测到锚蛋白缺乏,在1个家族中检测到带3蛋白(阴离子交换蛋白)缺乏。6个有锚蛋白缺乏的家族的患者,其锚蛋白2.1减少了19% - 51%,而有带3蛋白缺乏的家族该蛋白含量减少了33%。经学生t检验分析,所有变化均具有统计学意义(P<0.05)。溶血动力学分析能可靠地表明球形红细胞渗透特性的改变。