Blood, which is the only fluid tissue of the body in the circulatory system, performs very important tasks for the continuation of life. The most important task of blood tissue, which has a very complex structure, is to carry oxygen from the lungs to the cells and to ensure that it is removed from our body by carrying carbon dioxide from the cells to the lungs. Blood is responsible for transporting oxygen as well as nutrients, enzymes, hormones, etc. to the cells in our bodies.
Blood consists of many cells. While red blood cells (erythrocytes) are responsible for transporting oxygen, white blood cells (leukocytes) protect our body against microbes. Blood platelets (thrombocytes or platelets), which are the smallest cells of the blood, are responsible for stopping this bleeding when bleeding develops for any reason.
However, blood clotting is caused by a highly complex mechanism and a combination of many factors.
Hemophilia is a bleeding disorder that results in inadequate clotting of the blood. Patients with hemophilia may experience prolonged bleeding times or prolonged blood leakage after any trauma or surgical intervention. In patients with severe hemophilia, prolongation of bleeding time may occur after minor traumas, as well as spontaneous bleeding without any trauma. Serious bleeding may occur in the joint, muscles, brain or other organs.
The most common types of hemophilia are Hemophilia-A and Hemophilia-B.
- Hemophilia-A is also known as classical hemophilia or factor VIII deficiency, which is responsible for blood clotting. In other words, hemophilia-A patients have factor VIII deficiency.
- Hemophilia-B is also known as Christmas disease or factor IX deficiency. There is factor IX deficiency in hemophilia-B disease. Hemophilia-B, an atypical form of "hemophilia-B Leyden" disease, bleeding occurs mostly in childhood, these bleeding decreases after adolescence.
Although the symptoms of both types of hemophilia are very similar, the mutations responsible for the development of both types of hemophilia affect different genes and their treatments are different.
The basic principle in the treatment of hemophilia is to replace the missing factor and prevent bleeding.