Mild Hemophilia A and Its Response to DDAVP

Abstract

Abstract : Twenty-one patients with muld hemophilia A and their responses to DDAVP were reported. The initial bleeding episodes of ecchymosis, excessive bleeding after trauma and dental procedures occurred at the age of 3 months to 17 years with a mean age of 5 years, 6 months. The laboratory tests revealed prolonged activated partial thromboplastin time and low factor VII clotting activity (F VIII:C) of 5% to 24% (mean 9.6%). The patients received 94 episodes of intravenous DDAVP administration for clinical trials, preparation for dental procedures and treatment of bleeding episodes. However, the F VIII C levels before and after DDAVP administration were measured revealing good responses in 32 out of 33 episodes (97%). The median increment of F VIII C level 2.4 times over the baseline one hour after receiving medication. The median plasma half life of F VIIIC was seven hours. The bleeding symptoms were successfully controlled without requing any blood component and no adverse effect was found.
In addition, seven patients also received intranasal DDAVP administation for clinical trials. The median increment of F VIII:C level was 2 times over the baseline one hour after receiving medication. In conclusion, DDAVP is a useful altemative in the management of bleeding enisodes in mid hemophilia A. The intravenous form is sufficient for the treatment of bleeding episodes and preparation for simple surgery or dental procedures while the intranasal form is suitable for the first aid treatment of early bleeding episodes in home treatment.