Email updates

Keep up to date with the latest news and content from Journal of Medical Case Reports and BioMed Central.

Open Access Highly Accessed Case report

Successful immune tolerance induction consisting of high-dose factor VIII rich in von Willebrand factor and pulsed intravenous immunoglobulin: a case report

Peter Kubisz*, Ivana Plamenova, Pavol Holly and Jan Stasko

Author Affiliations

Department of Hematology and Transfusion Medicine, Jessenius Faculty of Medicine of the Comenius University and University Hospital Martin, Kollarova 2, Martin 036 59, Slovakia

For all author emails, please log on.

Journal of Medical Case Reports 2012, 6:350  doi:10.1186/1752-1947-6-350

Published: 11 October 2012

Abstract

Introduction

The development of factor VIII inhibitors is a serious complication of replacement therapy in patients with congenital hemophilia A. Immune tolerance induction has been accepted as the only clinically proven treatment allowing antigen-specific tolerance to factor VIII. However, some of its issues, such as patient selection, timing, factor VIII dosing, use of immunosuppressive or immunomodulatory procedures, still remain the subject of debate.

Case presentation

A case of a 3-year-old Caucasian boy with severe congenital hemophilia A, intron 22 inversion of the F8 gene and high-titer inhibitor, who underwent an immune tolerance induction according to the modified Bonn regimen (high doses of plasma-derived factor VIII rich in von Willebrand factor and pulsed intravenous immunoglobulin) is presented. The treatment lasted for 13 months and led to the eradication of inhibitor.

Conclusion

Addition of intravenous immunoglobulin did not negatively affect the course of immune tolerance induction and led to the rapid eradication of factor VIII inhibitor.

Keywords:
Coagulation factor VIII inhibitor; Hemophilia A; Immune tolerance induction; Intravenous immunoglobulin; Recombinant activated factor VII