[Leukocyte and thrombocyte glycosaminoglycans in hemophilia A and von Willebrand's disease].

AIM

The study of glycosaminoglycanes (GAG) in leukocytes and platelets of patients with hereditary coagulopathy.

MATERIALS AND METHODS

GAG concentration, composition and fraction identification were made in 25 patients with hemophilia A and 10 patients with Willebrand disease.

RESULTS

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.

CONCLUSION

Metabolism and/or release of GAG from blood cells may be involved in pathogenesis of hemophilia A and Willebrand disease.