In this condition, there is a considerably large deficiency of factor VIII.
The deficiency of factor VIII results in frequent spontaneous hemorrhage, as well as abnormal bleeding from minor injuries such as teeth extractions, and various surgeries.
In contrast to the mild and moderate forms of the condition, it is accompanied by spontaneous bleeding episodes.
This condition differs from Haemophilia A which is the most common clotting disorder caused due to deficiency of factor VIII.