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Hemophilias and Von Willebrand's Disease

Hemophilia A (factor VIII) and B (factor IX) are both X-linked recessive, coagulation factor disorders. In both hemophilia A and B a normal level of factor is present, but its coagulant activity is diminished. Hemophilia A is far more common. The clinical presentation varies according to the degree of coagulant activity present. In addition to coagulant activity, factor VIII stimulates platelet adhesion. When this function is diminished, von Willebrand's disease results.

Causes

Signs and Symptoms

Hemophilia

Hemarthrosis or muscle hematoma is most common. Chronic joint destruction can occur because of repetitive hemarthrosis often caused by minimal trauma.
Bleeding also can occur elsewhere, including the CNS, and may indicate more severe factor dysfunction. In severe cases (when factor activity is less than 1%), spontaneous hemorrhage is more common.

von Willebrand's disease

Generally less symptomatic than hemophilia
Epistaxis, excessive bruising, or prolonged bleeding after minor surgery (e.g., dental extractions) Hemarthroses or soft tissue bleeding, as seen with hemophilia, is less common.

Treatment

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