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




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Journal of Medical Case Reports

, 6:350

First Online: 11 October 2012Received: 18 April 2012Accepted: 10 September 2012DOI: 10.1186-1752-1947-6-350

Cite this article as: Kubisz, P., Plamenova, I., Holly, P. et al. J Med Case Reports 2012 6: 350. doi:10.1186-1752-1947-6-350

Abstract

IntroductionThe 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 presentationA 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.

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

KeywordsCoagulation factor VIII inhibitor Hemophilia A Immune tolerance induction Intravenous immunoglobulin Recombinant activated factor VII AbbreviationsBUBethesda unit

CVADCentral venous access device

FEIBAFactor VIII inhibitor bypass activity

FVIIIFactor VIII

ITInhibitor titer

ITIImmune tolerance induction

IUInternational unit

IVIntravenous

IVIGIntravenous immunoglobulin

rFVIIaRecombinant activated factor VII

vWFvon Willebrand factor.

Electronic supplementary materialThe online version of this article doi:10.1186-1752-1947-6-350 contains supplementary material, which is available to authorized users.

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Autor: Peter Kubisz - Ivana Plamenova - Pavol Holly - Jan Stasko

Fuente: https://link.springer.com/







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