[Leukocyte and thrombocyte glycosaminoglycans in hemophilia A and von Willebrand's disease].
OBJECTIVE
The study of glycosaminoglycanes (GAG) in leukocytes and platelets of patients with hereditary coagulopathy.


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.


CONCLUSIONS
Metabolism and/or release of GAG from blood cells may be involved in pathogenesis of hemophilia A and Willebrand disease.
Kharchenko
M F
MF
Egorova
L V
LV
Bitiukova
E S
ES
Tutova
I Iu
IIu
Nazarova
N S
NS
Papaian
L P
LP
Kargin
V D
VD
D000293:Adolescent; D000328:Adult; D001792:Blood Platelets / Q000737:chemistry*; D002469:Cell Separation / Q000379:methods; D002648:Child; D004588:Electrophoresis, Cellulose Acetate; D005260:Female; D006025:Glycosaminoglycans / Q000097:blood*; D006467:Hemophilia A / Q000097:blood* / Q000209:etiology; D006801:Humans; D007962:Leukocytes / Q000737:chemistry*; D008297:Male; D014842:von Willebrand Diseases / Q000097:blood* / Q000209:etiology
D006025:Glycosaminoglycans
Glikozaminoglikany leĭkotsitov i trombotsitov pri gemofilii A i bolezni Villebranda.