Kharchenko M F, Egorova L V, Bitiukova E S, Tutova I Iu, Nazarova N S, Papaian L P, Kargin V D
Ter Arkh. 1998;70(3):67-71.
The study of glycosaminoglycanes (GAG) in leukocytes and platelets of patients with hereditary coagulopathy.
GAG concentration, composition and fraction identification were made in 25 patients with hemophilia A and 10 patients with Willebrand disease.
In hemophiliacs, leukocytes contained low concentrations of GAG. In those with bleeding and synovitis GAG levels were lower than the average, in those with extensive hematomas in the absence of locomotor disorders the above levels were close to normal. Chondroitinsulphate dominated in GAG composition though it was less polydisperse. Heparin sulphate levels were elevated. Platelet GAG characteristics were close to normal. In Willebrand disease leukocyte GAG content and composition was similar to those in hemophilia A except some differences in electrophoretic properties of small GAG components.
Metabolism and/or release of GAG from blood cells may be involved in pathogenesis of hemophilia A and Willebrand disease.
研究遗传性凝血病患者白细胞和血小板中的糖胺聚糖（GAG）。
对25例甲型血友病患者和10例血管性血友病患者进行了GAG浓度、组成及组分鉴定。
血友病患者白细胞中GAG浓度较低。有出血和滑膜炎的患者GAG水平低于平均水平，无运动障碍但有广泛血肿的患者上述水平接近正常。GAG组成中硫酸软骨素占主导，但其多分散性较小。硫酸肝素水平升高。血小板GAG特征接近正常。血管性血友病患者白细胞GAG含量和组成与甲型血友病患者相似，只是小GAG组分的电泳性质存在一些差异。
血细胞中GAG的代谢和/或释放可能参与甲型血友病和血管性血友病的发病机制。
