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Meta-Analysis
. 2014 Aug;166(4):485-95.
doi: 10.1111/bjh.12942. Epub 2014 May 16.

Effect of von Willebrand factor on inhibitor eradication in patients with severe haemophilia A: a systematic review

Affiliations
Meta-Analysis

Effect of von Willebrand factor on inhibitor eradication in patients with severe haemophilia A: a systematic review

Alice S van Velzen et al. Br J Haematol. 2014 Aug.

Abstract

This systematic review was designed to summarize the reported valid quantitative evidence on the association between use of von Willebrand factor (VWF)-containing Factor VIII (FVIII) concentrates and successful immune tolerance induction (ITI) in patients with severe haemophilia A. The primary outcome was successful ITI; secondary outcomes were time to success, complications of the inhibitor or ITI and relapse of the inhibitor. A systematic literature search identified 26 randomized controlled trials, registries and cohort studies, evaluating a total of 1284 patients. For a pooled meta-analysis, 13 studies evaluating 382 patients were included. Due to incomplete data we were not able to assign pre-ITI risk categories to all patients for risk factor analysis. The meta-analysis did not demonstrate a difference in the proportion of patients with successful inhibitor eradication between those treated with VWF-containing products and those treated with FVIII concentrates devoid of VWF (relative risk [RR] 0·70 (95% confidence interval [CI] 0·52-0·89) and 0·84 (95% CI 0·75-0·93) respectively). Bleeding rate during ITI ranged from 0·00 to 0·85 bleeding episodes per year. The proportion of patients with a relapse of the inhibitor (range 0-20%) was mentioned in four studies that were included in the meta-analysis. The results of this systematic review do not support the idea of a positive effect of VWF-containing products in ITI.

Keywords: VWF; antibodies; haemophilia A; human factor VIII protein; immunology.

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