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Effect of von W illebrand factor on inhibitor eradication in patients with severe haemophilia A : a systematic review
Author(s) -
Velzen Alice S.,
Peters Marjolein,
Bom Johanna G.,
Fijnvandraat Karin
Publication year - 2014
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.12942
Subject(s) - medicine , meta analysis , haemophilia , von willebrand factor , confidence interval , relative risk , haemophilia a , randomized controlled trial , von willebrand disease , risk factor , gastroenterology , surgery , platelet
Summary 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 .