Congenital FVII deficiency and thrombotic events after replacement therapy

Thrombosis has been occasionally described in congenital FVII de\ufb01ciency. This report deals with patients with FVII de\ufb01ciency who presented thrombotic events after substitution therapy. At least 12 patients are reported in the literature. In all but two cases thrombosis occurred after prothrombin complex concentrates or plasma derived FVII concentrates. In two instances pulmonary embolism occurred after the administration of large amounts of whole blood. Concomitant prothrombotic risk factors were present in most of these cases (surgery, immobilization, old age, etc.). Personal \ufb01les allowed us to add another patient who developed bilateral pulmonary embolism after two vials of an aFVII concentrate. In this case also, concomitant risk factors were present, namely surgery for hysterectomy,immobilization.Thepulmonaryembolismoccurred in spite of the congenital FVII de\ufb01ciency indicating that no sure antithrombotic protection is assured by this defect. The actual needs of substitution therapy in patients with some variants of FVII de\ufb01ciency is discussed, together with comments on the therapeutic management of the thrombotic events in these patients