The severity of hemophilia B is determined by plasma coagulation factor IX activity and clinical symptoms, and is more serious if it is severe or combined with bleeding.
Hemophilia B is a coagulation disorder caused by coagulation factor IX deficiency or plasma thromboplastin deficiency. The main clinical manifestations are excessive bleeding or spontaneous bleeding after injury to weight-bearing joints, soft tissues and mucous membranes.
1. Mild: plasma coagulation factor IX activity is 5-25% of normal, clinical manifestations are post-surgical or post-injury bleeding, while spontaneous hematoma is extremely rare.
2. Moderate: plasma coagulation factor IX activity is 1-5% of normal, mostly manifested as post-surgical or post-injury bleeding.
3. Severe: plasma coagulation factor IX activity is less than 1% of normal, mostly manifested as spontaneous bleeding in early infancy and childhood, recurrent spontaneous hematoma and multiple bleeding, and coagulation factor IX substitution therapy is needed to survive for a longer period of time.
Hemophilia B severe or with severe bleeding is more serious, it is recommended to go to the hospital in time to give targeted treatment or treatment, so as not to delay the condition.