Thromb Haemost 2006; 96(02): 113-118
DOI: 10.1160/TH06-02-0078
Review Article
Schattauer GmbH

Inhibitors in mild/moderate haemophilia A: An update

Massimo Franchini
1  Servizio di Immunoematologia e Trasfusione– Centro Emofilia, Azienda Ospedaliera di Verona, Verona; Istituto di Chimica e Microscopia Clinica, Dipartimento di Scienze Biomediche e Morfologiche, Università di Verona, Verona, Italy
,
Gian Luca Salvagno
1  Servizio di Immunoematologia e Trasfusione– Centro Emofilia, Azienda Ospedaliera di Verona, Verona; Istituto di Chimica e Microscopia Clinica, Dipartimento di Scienze Biomediche e Morfologiche, Università di Verona, Verona, Italy
,
Giuseppe Lippi
1  Servizio di Immunoematologia e Trasfusione– Centro Emofilia, Azienda Ospedaliera di Verona, Verona; Istituto di Chimica e Microscopia Clinica, Dipartimento di Scienze Biomediche e Morfologiche, Università di Verona, Verona, Italy
› Author Affiliations
Further Information

Publication History

Received 09 February 2006

Accepted after resubmission 26 June 2006

Publication Date:
28 November 2017 (online)

Summary

The development of inhibitors in patients with mild/moderate hemophilia A is an increasingly recognized occurrence and is manifested by the patients’ bleeding pattern becoming more severe. Inherited (hemophilia genetic mutations) and acquired (type and delivery of factor VIII replacement therapy) factors have been associated with an increased likelihood of developing factor VIII inhibitors. Although the use of bypassing agents (i.e. activated prothrombin complex concentrates and recombinant factor VII activated) has been demonstrated to be effective in controlling bleeding episodes in patients who develop factor VIII inhibitors, the limited data available in the literature are insufficient to determine the optimal approach to the eradication of inhibitors (i.e. immune tolerance induction, immunosuppression or both) for this group. Particular attention should be directed to the prevention of this complication in those patients with mild/moderate hemophilia recognized to be at increased risk of developing a factor VIII inhibitor. In conclusion, large prospective trials are warranted in order to elucidate the many still unclear pathogenic and therapeutic aspects of the development of inhibitors in patients with mild/moderate hemophilia A.