2003; baxter - Supplements - Haematologica

[Round Table on Immune Tolerance Treatment]review paperThe Malmö International BrotherStudy (MIBS) – An updatehaematologica 2003; 88(suppl. n. 12):45-47http://www.haematologica.org/free/immunotolerance2001.pdfJAN ASTERMARK, ERIK BERNTORP AND THE MIBS STUDY GROUP*Department for Coagulation Disorders, University of Lund,Malmö, SwedenThe Malmö International Brother Study (MIBS) wasinitiated in 1996 to study the issue of predisposingfactors to inhibitor development in hemophiliapatients. As of August 2001, 528 families haveaccrued, of whom 451 suffer from hemophilia A and77 hemophilia B. Twenty-five of the brother pairs aretwins. The inhibitor incidence in all families withsevere hemophilia A was 29.8%. In 36 of the 110inhibitor families (32.7%), at least two brothers hada history of inhibitors. In 24 of these families, theinhibitor was also of the same type, i.e. either highorlow-responding. The overall concordance within249 severe hemophilia A families was found to be78.3% compared to an expected figure of 68.0%and 58.0% using an inhibitor incidence of 20 and30%, respectively (p5 BU and a low-respondinginhibitor was one with a peak titer of ≤ 5 BU.Severe hemophilia A corresponds to a factor VIIIclotting activity of 5%, respectively.Statisticsχ 2 analysis was used for statistical evaluationof expected versus observed frequencies ofinhibitors. An expected number of inhibitor subjectsof at least five was required in each group.A p value below 0.05 was considered to indicatestatistical significance.haematologica vol. 88(supplement n. 12):september 2003