Fibrinogenmängel

Fibrinogenmangel, also known as hypofibrinogenemia or afibrinogenemia, refers to a group of inherited bleeding disorders characterized by a deficiency or absence of fibrinogen in the blood. Fibrinogen is a crucial protein produced by the liver that plays a vital role in blood clotting. When blood is injured, fibrinogen is converted into fibrin, which forms a mesh-like structure that traps blood cells and platelets, creating a clot to stop bleeding.

There are two main types of fibrinogenmangel: quantitative and qualitative. Quantitative disorders involve a reduced amount of fibrinogen, while qualitative disorders involve the production of abnormal fibrinogen molecules that are dysfunctional. The severity of the condition can vary significantly, ranging from mild bleeding tendencies to severe, life-threatening hemorrhages. Symptoms may include easy bruising, nosebleeds, prolonged bleeding after injury or surgery, and internal bleeding. In women, heavy menstrual bleeding and bleeding after childbirth can also occur.

Diagnosis typically involves blood tests that measure fibrinogen levels and assess its clotting function. Genetic testing may be used to identify the specific mutation responsible for the deficiency. Treatment strategies depend on the severity and type of fibrinogenmangel. For mild cases, observation may be sufficient. For more significant bleeding, treatment involves replacing the missing fibrinogen with commercially available fibrinogen concentrates or fresh frozen plasma. Regular monitoring and prophylactic treatment may be necessary for individuals with severe forms of the disorder to prevent serious bleeding events.