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Table 2 Some molecular and physiological characteristics of hemophilia A and B

From: Advanced therapies for the treatment of hemophilia: future perspectives

Type of hemophilia

Deficient factor

Protein size (Kilodaltons)

DNA length (bp)

Half life (Hours)

Function in the coagulation cascade

Plasma concentration (ng/ml)1,2

      

Mild hemophilia

Moderately Severe Hemophilia

Severe Hemophilia

Hemophilia A

VIII

280

7056

8-12

Activated by thrombin and activates to FX3

10-80 (5-40%)

2-10 (1-5%)

< 2 (<1%)

Hemophilia B

IX

68

1389

15-25

Activated by FXIa4, and activates to FX

500-2000 (5-40%)

50-500 (1-5%)

< 50 (<1%)

  1. 1Normal plasma level of coagulation factors: Factor VIII, 200 ng/mL; factor IX, 5000 ng/mL; 2One International Unit (IU) of factor VIII or IX, per kg of body weight, increases the activity of factor in plasma, 1% to 2% of normal level; 3FX, factor X; 4FXIa, factor XI activated.