Azadeh Farjami - Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
Sezaneh Haghpanah - Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
Peyman Arasteh - Noncommunicable Diseases Research Center, Fasa University of Medical Sciences, Fasa, Iran
Rezvan Ardeshiri - Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
Hakimeh Tavoosi - Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
Zohre Zahedi - Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
Shirin Parand - Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
Mehran Karimi - Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran

Background: Data on the frequency of hereditary bleeding disorders (HBDs) and associated mortality and morbidities during a long-term follow-up from Iran are scarce. Objective: This study evaluated the epidemiologic features among patients with HBD in one of the largest referral centers in southern Iran. Methods: In this cross-sectional study, ۶۱۹ patients with HBD were evaluated during the period ۱۹۹۶ to ۲۰۱۱. Aside from baseline characteristics and type of factor deficiency, associated morbidities including viral infections, neurological disorders, asthma, thalassemia, glucose-۶-phosphate dehydrogenase (G۶PD) deficiency, diabetes, hypertension, cardiac and renal diseases were evaluated. Furthermore, among patients who died, the underlying disease and etiology of death were also evaluated. Results: Patients’ mean age was ۲۴.۴ ± ۱۳.۵ years. Factor VIII deficiency was the most prevalent type (۵۰.۴%) of HBD, and combined Von–Willebrand and factor XIII deficiency (۲.۳%) was the most prevalent type of combined factor deficiency. A total of ۰.۵% had hepatitis B and ۱۱.۵% had hepatitis C. Cardiac disease was seen in ۱.۵%, hypertension in ۰.۲%, renal disease in ۰.۲%, and diabetes in ۱.۳% of patients. Overall, ۵.۲% had intracranial hemorrhage, ۲.۱% had epilepsy, and ۰.۸% had mental retardation. During the ۱۵-year follow-up, ۲۲ patients died; car accident was the leading cause of death in this population. Conclusion: Associated morbidities were seen in ۲۴.۳% of patients with HBD. Most prevalent morbidities were HCV infections (۱۱.۵%) and neurological disease (۷.۳%). The mortality rate among patients with HBD was ۳.۴%, and the most common cause of death was accident, which is similar to that of normal Iranian populations.

Hereditary Bleeding Disorder, Morbidity, mortality, Hemophilia