Document Type: Original Article
Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
Noncommunicable Diseases Research Center, Fasa University of Medical Sciences, Fasa, Iran
Department of MPH, 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, 619 patients with HBD were evaluated during the period 1996 to 2011. Aside from baseline characteristics and type of factor deficiency, associated morbidities including viral infections, neurological disorders, asthma, thalassemia, glucose-6-phosphate dehydrogenase (G6PD) 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 24.4 ± 13.5 years. Factor VIII deficiency was the most prevalent type (50.4%) of HBD, and combined Von–Willebrand and factor XIII deficiency (2.3%) was the most prevalent type of combined factor deficiency. A total of 0.5% had hepatitis B and 11.5% had hepatitis C. Cardiac disease was seen in 1.5%, hypertension in 0.2%, renal disease in 0.2%, and diabetes in 1.3% of patients. Overall, 5.2% had intracranial hemorrhage, 2.1% had epilepsy, and 0.8% had mental retardation. During the 15-year follow-up, 22 patients died; car accident was the leading cause of death in this population.
Conclusion: Associated morbidities were seen in 24.3% of patients with HBD. Most prevalent morbidities were HCV infections (11.5%) and neurological disease (7.3%). The mortality rate among patients with HBD was 3.4%, and the most common cause of death was accident, which is similar to that of normal Iranian populations.