Leukocyte and thrombocyte glycosaminoglycans in hemophilia a and von Willebrand's disease
Kharchenko, M.F.; Egorova, L.V.; Bitiukova, E.S.; Tutova, I.I.; Nazarova, N.S.; Papaian, L.P.; Kargin, V.D.
Terapevticheskii Arkhiv 70(3): 67-71
1998
ISSN/ISBN: 0040-3660 PMID: 9575593 Document Number: 488383
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.