Willebrandszindróma

Willebrandszindróma refers to a group of inherited bleeding disorders characterized by defects in von Willebrand factor (vWF). Von Willebrand factor is a protein that plays a crucial role in blood clotting by helping platelets stick to the site of an injury and by protecting factor VIII, another important clotting protein. This syndrome is the most common inherited bleeding disorder worldwide, affecting both men and women.

There are several types of Willebrandszindróma, primarily categorized into quantitative and qualitative defects. Type 1 is the most common and involves a reduced amount of vWF. Type 2 encompasses qualitative defects, where the vWF is present in normal or near-normal amounts but is dysfunctional. Type 3 is the rarest and most severe, characterized by a near-complete absence of vWF. Acquired forms of Willebrandszindróma can also occur, often associated with other medical conditions.

Symptoms can vary widely in severity, ranging from mild to life-threatening. Common manifestations include easy bruising, prolonged bleeding after injuries or surgery, nosebleeds, and heavy or prolonged menstrual periods in women. Gastrointestinal bleeding and joint bleeding can also occur. Diagnosis is made through a combination of clinical evaluation and laboratory tests that measure vWF levels and function, as well as factor VIII activity. Treatment strategies depend on the specific type and severity of the syndrome and may involve medications to increase vWF levels, replacement therapy with vWF-containing products, or drugs to stop bleeding.