LAUSR

Objective To identify the data related with therapeutic and patients characteristics and their correlation with the inhibitor’s presence in hemophilia A in Pediatric Hematologic/Oncologic Unit. University Hospital Centre ‘Mother Teresa’, Tirana, Albania, the unique national service that treat pediatric coagulopathies. Design This analytic, observational, retrospective and case – control study analyses 32 patients (0–14 years old) diagnosed and treated in our center for hemophilia A, that had been tested for inhibitor presence (32 from 45 children with hemophilia A in Albania). Outcome measures Inhibitor’s presence (qualitative test) after 50 days of exposure, severity of the disease, breastfeeding, family history for hemophilia, age and purpose of first FVIII exposure, age of 50th exposure, time between first and 50th exposure, surgery up to 50th day of exposure, type and number of exposures episode (minor or major) and FVIII average dosage. Results Inhibitors were present in 13 from 32 patients. The mean age of the first exposure to FVIII is 20.1 months old and in 25 of 32 patients hemorrhage was the reason for the first exposure. There was no significant statistical difference between inhibitors presence and age and reasons of first exposure. Neither nor the age of the 50th exposure wasn’t correlated with inhibitors presence. The mean age between first and 50th day of exposure was 24.2 months and there was a positive correlation between this data and inhibitors presence (t-Test = 2.267; df = 30; p = 0.031), also the number of episodes of exposition until the 50th day of exposition (avg.6.5) was correlated with inhibitors presence (t-Test = 3.270; df = 30; p = 0.003). We had more major exposure episodes (78.1%) and this data influenced in inhibitor’s presence (Fisher’s Test = 6.131; df = 1; p = 0.015). The average dosage of FVIII was also correlated with inhibitor’s presence (χ2=12,799; df = 2; p = 0.002). Severity of the disease and family history for hemophilia weren’t correlated with the inhibitors. Breastfeeding with associated with lower risk for inhibitors presence (t=7.166;df=;p=0.011). Conslusions Inhibitor’s presence percentage in Albanian pediatric patients with hemophilia A is 40.6%. This data is much higher than the literature reports. Factors influencing more in the development or presence of inhibitors in our population are: high dosage of factor VIII, the high frequency of exposure episodes and the short time between first and the 50th exposure day.