GLYCOSAMINOGLYCANES OF LEUKOCYTES AND PLA TELETS IN HEMOPHILIA-A AND VON-WILLEBRAND-DISEASE

Aim. The study of glycosaminoglycanes (GAG) in leukocytes and platelet s of patients with hereditary coagulopathy. Materials and Methods. GAG concentration, composition and fraction identification were made in 2 5 patients with hemophilia A and 10 patients with Willebrand disease. Results. In hemophiliacs, leukocytes contained low concentrations of G AG. In those with bleeding and synovitis GAG levels were lower than th e average, in those with extensive hematomas in the absence of locomot or disorders the above levels were close to normal. Chondroitinsulphat e dominated in GAG composition though it was less polydispurse. Hepari n sulphate levels were elevated Platelet GAG characteristics were clos e to normal. In Willenbrand disease leukocyte GAG content and composit ion was similar to those in hemophilia A except some differences in el ectrophoretic properties of small GAG components. Conclusion. Metaboli sm and/or release of GAG from blood cells may be involved in pathogene sis of hemophilia A and Willebrand disease.