hüpofibrinogeneemia

Hypofibrinogenemia is a rare bleeding disorder characterized by low levels of fibrinogen in the blood. Fibrinogen, also known as Factor I, is a protein essential for blood clotting. When a blood vessel is injured, fibrinogen is converted into fibrin, which forms a mesh that traps blood cells and stops bleeding. In individuals with hypofibrinogenemia, the insufficient amount of fibrinogen impairs the ability of blood to clot effectively.

This condition can be inherited, meaning it is passed down through families, or it can be acquired due to other medical conditions or treatments. Inherited hypofibrinogenemia is typically caused by mutations in the genes responsible for producing fibrinogen. Acquired forms can result from liver disease, malnutrition, disseminated intravascular coagulation (DIC), or certain medications.

Symptoms of hypofibrinogenemia can vary in severity. Mild cases may present with easy bruising, prolonged bleeding after injury or surgery, and nosebleeds. More severe cases can lead to spontaneous or excessive bleeding, particularly into joints and muscles, and may even cause life-threatening hemorrhages. Diagnosis is made through blood tests that measure fibrinogen levels. Treatment focuses on managing bleeding episodes and may involve the administration of fibrinogen concentrate or fresh frozen plasma. Individuals with this condition often require lifelong monitoring and management by a hematologist.