Alternations of oxidative stress markers in patients with severe haemophilia treated by different prophylaxis regimens

Abstract

Background: Due to recurrent bleding in haemophilia patients, the effect of blood, i.e. free iron in the joint spaces, could initiate releasing markers of oxidative stress in physiological fluids. Aims: The aim of this study were to estimate the influence of differ- ent prophylaxis regimens for severe haemophilia on alternations of oxidative stress markers in serum: a) advanced oxidation protein products (AOPP), b) paraoxonase-1 (PON1) and c) sulfhydril groups (SHG). Methods: The study included 20 adult patients with severe haemo- philia. Five patients with haemophilia A received prophylaxis with FVIII concentrate in the standard dose of 20 IU kg 1 three times per week, while another five patients with haemophilia A were given an intermediate dose of FVIII - 10–15 IU kg 1 thrice weekly. Seven patients with haemophilia A and three with haemophilia B, received FVIII/IX concentrate only on-demand. The following oxidative stress markers were measured: a) AOPP, b) PON1 and c) SHG. Serum sam- ples were collected initially, before the start of treatment (labelled AOPP-1, PON1-1 and SHG-1) and after 3 months follow-up (labelled AOPP-2, PON1-2 and SHG-2). Results: The mean age of the patients was 32 years (range 19–55). In the group of patients given standard dose prophylaxis, the mean values of AOPP-2 (P = 0.018), PON1-2 (P = 0.043) and SHG-2 (P = 0.045) were significantly lower than those for AOPP-1, PON1- 1 and SHG-1. Likewise, the mean values for AOPP-2 (P = 0.047) and PON1-2 (P = 0.039) in the five patients receiving intermediate dose prophylaxis were also decreased when compared to initial val- ues, but SHG level was not significantly changed. In patients trea- ted on demand the mean values for AOPP, PON1 and SHG did not alter significantly. Conclusion: The results of this investigation showed that treatment of patients with severe haemophilia by continuous prophylaxis with intravenously applied FVIII/IX provides less oxidative stress reflected as decreasing of markers such as AOPP, PON1 and SHG.