The use of intravenous immunoglobulin in immune tolerance induction in inherited haemophilia A: a single-centre experience and a review of literature.

The immune tolerance induction is the treatment of choice for the eradication of factor VIII inhibitors, a serious complication of inherited haemophilia A. Despite the preferred treatment of patients with good prognosis and testing of different regimens, the immune tolerance is achieved in 70-80%. Several modifications of regimens including the addition of immunomodulatory agents were proposed in order to improve immune tolerance induction (ITI) outcome. Intravenous immunoglobulin has complex immunomodulatory properties and has been used in immune tolerance induction since the introduction of Malmö regimen in the 1980s. The aim of the work is to evaluate the published evidence of its use in ITI in haemophilia A. In addition, the authors' own experience with a high-dose regimen using human plasma-derived factor VIII containing von Willebrand factor and pulsed IVIg is reported. The course of three patients with severe inherited haemophilia A and inhibitor (all high responders, two with poor prognosis, one with rescue treatment) is described. At least partial success was achieved in all patients and no adverse events attributable to intravenous immunoglobulin were observed. Despite the limited evidence, the addition of intravenous immunoglobulin appears to be a promising treatment modification for patients with poor prognosis or previous failure of immune tolerance induction.

Citation

Peter Kubisz, Pavol Hollý, Ján Staško, Ivana Plameňová. The use of intravenous immunoglobulin in immune tolerance induction in inherited haemophilia A: a single-centre experience and a review of literature. Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis. 2015 Sep;26(6):604-12

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PMID: 25886836

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