Autori: M. Mihailov, M. Serban, D. Savescu, S. Arghirescu, C. Petrescu, M. Puiu;
Editorial: nature publishing group, European journal of human genetics, Volume 16 Supplement 2 May 2008, p.316, 2008.
Introduction. FVIII inhibitors appearance in haemophiliacs is related to several factors, like substitution with native plasma products, frequent changing of FVIII concentrates, high rate of HBV and HCV infection, pseudotumours and other massive muscular haematomas, etc.
Objective. Starting from particularities of haemophilia treatment in our country, we evaluated the variables impact on inhibitors development. We assessed frequency and inhibitors titer, in correlation with risk factors.
Material and method: retrospective single center study based on 219 hemophilia patients registered and treated in the IIIrd Pediatric Clinic Timisoara, using Bethesda assay. We evaluated the influence of some parameters on frequency and inhibitors titer: haemophilia severity, gene mutations, family history, type of substitutive product used, hepatitis C or B infection, Interferon alpha therapy, surgical interventions, and bolus vs. intravenous infusion of FVIII.
Results. Between January 1997- 2002, inhibitors frequency was 47.5%, whereas after January 2002 only 19.19% of patients had inhibitors due to reactants and methodology change. The majority of inhibitors were in severe haemophilia (78.95%), 15 of these patients being positive for intron 22 inversion.
Family history and exposure to FVIII were important risk factors of inhibitors development. Hepatitis C or B infection, Interferon alpha therapy, surgical interventions and intravenous infusion of FVIII haven’t been significantly correlated with inhibitors’ development.
Conclusions. In our patients, we found only the following risk factors for inhibitors development: exposure to FVIII, type of FVIII gene mutations and familial predisposition. Accuracy of technique and experience of laboratory technician is decisive for inhibitor evaluation.
Cuvinte cheie: hemofilie, dezvoltare de inhibitori, risc // Inhibitors development*haemophilia*risk