ASSOCIATION OF SEVERE HEMOPHILIA-A AND FACTOR-V LEIDEN - REPORT OF 3 CASES

The clinical severity of haemophilia is usually related to residual cl otting factor activity. Reports of factor V deficiency or protein C ha ve been described in haemophilic patients and both are involved in the activated protein C pathway. Recently, the poor response to activated protein C due to factor V Leiden (FQ506) has been associated with an increased risk of thrombosis and its incidence in the normal populatio n is given as 2-7%. We describe three cases of severe haemophilia A he terozygous for factor V Leiden and its influence on phenotype.