Hemorrhagic diseases and syndromes - forms of a pathology at which the raised predilection to a staxis is marked. Distinguish the hereditary and got forms of hemorrhagic diseases. Hereditary forms - are bound to hereditary changes: a vascular wall; anomalies of megacaryocytes and thrombocytes; adhesive fibers of a blood plasma; plasma factors of coagulating system of a blood. The majority of the got forms of a staxis is bound with: immune, toxi-infectious and metabolic lesions of blood vessels; various damages of megacaryocytes, thrombocytes; the got disturbances of adhesive molecules of a blood plasma and factors of coagulating system of a blood; multifactorial damages to system of a blood. On a pathogenesis distinguish following groups of hemorrhagic diseases and syndromes: 1) caused by a primary lesion of a vascular wall with possible secondary involving in process of coagulative mechanisms of a hemostasis; 2) caused by thrombocytopenias; 3) caused by disturbances of coagulability of a blood at hemophilias, disturbance of stabilization of the fibrin, the raised fibrinolysis, including at treatment by direct and indirect anticoagulants, Streptokinasa, a urokinase and others; 4) caused by complex disturbances of various parts of system of a blood.
Pathogenesis. At children's or youthful age thin-walled, easily bleeding and injured small angiomas are formed; in some cases arteriovenous shunts in easy and other organs, other attributes of inferiority of a mesenchyma - an extensibility of a skin (a rubber skin), a relaxation of ligaments (habitual dislocations) are formed.
Clinical picture. Relapsing bleedings from the teleangiectasias located more often in a nasal cavity prevail. Bleed teleangiectasias on edge of labiums, mucosas of a mouth, pharynx, a stomach less often. The number of teleangiectasias and bleedings accrues during puberty and in the age of till 20-30 years. At presence of arteriovenous shunts there is a dyspnea, a cyanosis; by means of a roentgen in lungs find out individual spherical or the wrong form the shadows quite often wrongly accepted for tumours.
