ABSTRACT
We report on the data collected in the Italian Registry of acquired haemophilia (AH) in 2001. Recombinant activated factor VII (rFVIIa) was selected as first-line therapy in 19 bleeding episodes because of their severity and as salvage in one case. Bleeding was controlled in 90% of the episodes, indicating the efficacy of rFVIIa in HA.
Subject(s)
Factor VIII/immunology , Factor VII/therapeutic use , Hemophilia A/drug therapy , Autoantibodies , Factor VIIa , Hemophilia A/immunology , Hemorrhage/drug therapy , Hemorrhage/prevention & control , Humans , Italy , Recombinant Proteins/therapeutic use , RegistriesABSTRACT
OBJECTIVE: To review the clinical problems related to the inhibitor of factor VIII (FVIII) in pregnancy. DESIGN: Retrospective analysis. SETTING: Haemophilia and haemotology centres. POPULATION: Patients registered and followed up at the centres. METHODS: Data were collected from the Italian Haemophilia Register of acquired FVIII inhibitor. RESULTS: Twenty of 96 cases with FVIII inhibitor were identified postpartum. The time of appearance was 3-150 days postpartum. All but one of the cases were idiopathic; 11/20 patients required blood transfusions. In six patients, the inhibitor was identified because of surgical bleeding, four after hysterectomies carried out because of postpartum haemorrhage. A prolonged activated partial thromboplastin time was present in all women in whom the test was carried out. Nine women did not require treatment because the bleeding was mild; in 11 patients bleeding was promptly controlled by different therapeutic modalities. Immunosuppressive therapy was used to suppress the inhibitor. The majority of the patients who achieved complete remission received steroids; in 6/6 patients who relapsed, a second remission was obtained with combined therapy. CONCLUSIONS: In a review of 20 pregnancies with FVIII inhibitor, over a 15 year period, bleeding was controlled in all cases with no fatalities. Correct evaluation of coagulation screening tests, in particular, activated partial thromboplastin time, is essential.
