Utilization evaluation of factor concentration and frequencyof bleeds among patients with haemophilia "A" and haemophilia "B" in northwest Iran

Abstract

Background Haemophilia A and B are X-linked bleeding disorders which result in decreased blood levels of coagulants. According to some studies, Hemophilia Severity Score (HSS) is higher in severe Haemophilia A(HA) than in severe Haemophilia B(HB). Objectives The aim of this study was comparison of bleed frequency and utilization of factor concentrate between HA and HB patients. Materials/Patients and Methods This is a single institution retrospective study, and we gathered information from records of our Hemophilia Clinic .Our samples consisted of 176 Haemophilia A and 35 Haemophilia B severe and moderate deficient patients. All of our patients used on-demand treatment with plasma derived factor concentrates. Chi-Square , one sample T and Mann-Whitney U tests were used. All the calculations were performed with MedCalc Statistical Software 12.1.4 version. Results Overall admission rates for patients with Haemophilia A were 3.125/patient/year and for Haemophilia B were 0.77/patient/year (P<0.05).The amount of factor concentrates used by our HA patient was 3731500IU of FVIII (21201.704 IU/patient/year), and 611000 IU of Factor IX , by patients with hemophilia B(17457.142 IU/patient/year).The difference in the usage of factor concentrate was not statistically significant (P=0.57). Conclusion The data suggest that the two inherited coagulation disorders (Haemophilia A and Haemophilia B) have a different severity of clinical phenotype. Our findings correlate with findings by some other similar studies that have been published recently.

Keywords

• Hemophilia A
• Hemophilia B
• Factor Concentrate
• Utilization Evaluation