Rituximab in the Treatment of Postpartum Acquired Hemophilia A

Review Article

Rituximab in the Treatment of Postpartum Acquired Hemophilia A

Maria Gabriella Mazzucconi, Francesca Biondo and Cristina Santoro

Affiliation: Haematology Institute, Dipartimento di Biotecnologie cellulari ed Ematologia, ‘‘Sapienza’’ University of Rome, Italy

Submission date: 10th July 2009, Revision date: 2nd September 2009, Acceptance date: 14th September 2009


ABSTRACT

Acquired hemophilia A is a rare disorder caused by the development of autoantibodies against coagulation factor VIII: it is often characterized by severe bleeding tendency. Its incidence is 0.2–1 per million persons per year. It may be associated with malignancies, autoimmune disorders, or pregnancy but, in about 50% of cases, no cause can be identified. Postpartum acquired hemophilia A represents 7–21% of all cases, with an incidence of about 1/350 000 deliveries. The aim of the management of acquired hemophilia A and, in particular of postpartum acquired hemophilia A, is the control of bleeding, if present, and the eradication of the inhibitor. The latter can be obtained with immunosuppressive drugs. However, cyclophosphamide and other alkylating agents should not be used in young women with postpartum inhibitor because of the risk of infertility. Complete remission rate obtained with these agents is about 80% or more but, in some cases, persistence of the inhibitor despite treatment or relapse after response represent an actual risk of recurrent bleedings. Therefore, alternative therapeutic approaches are advisable. Rituximab has been used in acquired hemophilia A since 2001 either as first‐line therapy or in resistant/relapsed patients. Its efficacy is very high (about 90% eradication rate); it is safe and well tolerated, especially in young people. For these reasons, Rituximab can play a role in the therapeutic approach to postpartum acquired hemophilia A. Few cases have been treated up to now, but the results are very promising. Inhibitor eradication was obtained in all cases. No relevant side‐effects were noted, nor did infections occur. Cytotoxic immunosuppressive drugs can compromise fertility in young female patients, whereas Rituximab can be a valid and safe alternative treatment.

Keywords: acquired hemophilia A, inhibitor to factor VIII, immunosuppressive therapy, postpartum factor VIII inhibitor, Rituximab
Correspondence: Maria Gabriella Mazzucconi , Associate Professor, Dipartimento di Biotecnologie cellulari ed Ematologia, “Sapienza” University of Rome, via Benevento n. 6, 00161, Roma, Italy