Abstract

Our study was designed to describe the correlation between patient and therapy characteristics with inhibitors presence in Albanian pediatric patients who suffer from hemophilia A (previously treated more than 50 days with FVIII). We investigated all the pediatric (0-14 years old) patients of Albania with hemophilia A (45 patients were initially enrolled). The main outcome was the inhibitor presence, confirmed by our laboratory with qualitative test. The test was performed only in 32 of our patients (71% of population). Thirteen of them (40.6%) are positive for inhibitors presence; this finding is higher than the percentage, which is referred in other countries. We found an association between intensity of replacement therapy (time between first treatment with FVIII and the 50^th treatment) (t=2.267; df=30; p=0.031), frequency of treatment until the 50^th exposure day (t=3.270; df=30; p=0.003) and the average dosage of FVIII (χ²=12,799; df=2; p=0.002) with inhibitor presence. Also the length of exposure episode (more than 5 days) was correlated with the inhibitor presence (t=6.131; df=1; p=0.015). Breastfeeding was associated with lower risk for inhibitors presence (t=7.166; df=; p=0.011). In our study the severity of the disease, family history for hemophilia, age, reason of the first exposure and surgical treatment during the first 50 days of exposure were not significant statistical correlated. Our findings suggest that the aggressivity (intensity, frequency, dosage) of the replacement therapy with exogenous FVIII have a primary role in inhibitors presence or development.