Inhibitors in mild/moderate haemophilia A: An update

Massimo Franchini; Gian Luca Salvagno; Giuseppe Lippi

doi:10.1160/TH06-02-0078

Thrombosis and Haemostasis, Table of Contents

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

¹Servizio 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

¹Servizio 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

¹Servizio 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

Abstract

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.

Keywords

Inhibitors - bleeding - mild hemophilia A

Full Text

References

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