Hemophilia Thrombasthenic

Hemophilia or Glansen's thrombosthenia (from the Greek hemos - blood and stenos - narrow passage) is a hereditary disease that is characterized by impaired blood clotting and frequent bleeding. This disease is a consequence of a hereditary pathology, namely hematopoiesis.

Hemophilia and thrombosthenia are different forms of the same disease. They have a number of similar features, however, despite this, differences between them exist; in addition, both terms have a genetic component.

Thrombosthenia (synonym - Glanzmann-Mauriac-Savaliere hemophilia) is a hereditary disease of the hematopoietic system, characterized by a decrease in blood clotting. A distinctive feature of hemophilia A from other types of hemophilia is its familial nature, which indicates a different pathogenetic basis of the disease. Thus, the pathology is characterized by a mutation of the gene on the X chromosome, which is responsible for blood clotting processes. Leads to disruption of the production of factor VIII. Outwardly, this manifests itself in a decrease in the amount of blood in the arteries and small hemorrhages.

The main clinical sign of hemophilia is bleeding with the formation of hematomas, which occurs after minor trauma and can be easily stopped. Treatment should be started immediately after the first symptoms of the disease are identified, since it is later complicated by the appearance of defects in other organs. Great attention should also be paid to the preventive treatment of thrombosis.