An 18-year-old patient has been ill since early childhood. During the examination it was found: Hb - 110 g/l, erythrocytes - 3,9 × 1012/ l, KP - 0,8, leukocytes - b, 0 × 10 ^ g/l; ESR - 30 mm/year. Coagulogram showed: prothrombin index - 95%, blood clot retraction - 50%,blood clotting time - did not occur in 40 minutes, bleeding duration - 3 minutes. What mechanisms form the basis of the pathogenesis of this disease?