Document Type : Original Article


1 Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran

2 Noncommunicable Diseases Research Center, Fasa University of Medical Sciences, Fasa, Iran

3 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.


  1. Khleif AA, Rodriguez N, Brown D, Escobar MA. Multiple comorbid conditions among middle-aged and elderly hemophilia patients: prevalence estimates and implications for future care. J Aging Res. 2011;2011:985703. doi: 10.4061/2011/985703.
  2. Assarehzadegan MA, Ghafourian Boroujerdnia M, Zandian K. Prevalence of hepatitis B and C infections and HCV genotypes among haemophilia patients in haves, southwest Iran. Iran Red Crescent Med J. 2012;14(8):470-474.
  3. Yazdani MR, Kassaian N, Ataei B, Nokhodian Z, Adibi P. Hepatitis C virus infection in patients with hemophilia in Isfahan, Iran. Int J Prev Med. 2012;3(suppl 1):S89-93.
  4. Zhang M, Rosenberg PS, Brown DL, et al. Correlates of spontaneous clearance of hepatitis C virus among people with hemophilia. Blood. 2006;107(3):892-897. doi:10.1182/ blood-2005-07-2781.
  5. Gadalla SM, Preiss LR, Eyster ME, Goedert JJ. Correlates of high hepatitis C virus RNA load in a cohort of HIV-negative and HIV-positive individuals with haemophilia. J Viral Hepat. 2011;18(3):161-169. doi: 0.1111/j.1365-2893.2010.01289.x.
  6. Arababadi MK, Nasiri Ahmadabadi B, Yousefi Daredor H, Kennedy D. Epidemiology of occult hepatitis B infection among thalassemic, hemophilia, and hemodialysis patients. Hepat Mon. 2012;12(5):315-319. doi: 10.5812/hepatmon.5934.
  7. Lim MY, Pruthi RK. Cardiovascular disease risk factors: prevalence and management in adult hemophilia patients. Blood Coagul Fibrinolysis. 2011;22(5):402-406. doi:10.1097/ MBC.0b013e328345f582.
  8. Kulkarni R, Soucie JM, Evatt BL. Prevalence and risk factors for heart disease among males with hemophilia. Am J Hematol. 2005;79(1):36-42. doi: 10.1002/ajh.20339.
  9. Ranta S, Valta H, Viljakainen H, Makitie O, Makipernaa A. Hypercalciuria and kidney function in children with haemophilia. Haemophilia. 2013;19(2):200-205. doi:10.1111/ hae.12021.
  10. Ljung RC. Intracranial haemorrhage in haemophilia A and B. Br J Haematol. 2008;140(4):378-384. doi:10.1111/j.1365- 2141.2007.06949.x.
  11. Sharifi-Mood B, Eshghi P, Sanei-Moghaddam E, Hashemi M. Hepatitis B and C virus infections in patients with hemophilia in Zahedan, southeast Iran. Saudi Med J. 2007;28(10):1516- 1519.
  12. Karimi M, Hashemi A, Ghiam AF, Jahromi SS, Toobaee S. Substance dependency in Iranian patients with hemophilia. Addict Behav. 2007;32(2):365-369. doi: 10.1016/j.addbeh.2006.03.040.
  13. Alavian SM, Ardeshiri A, Hajarizadeh B. Prevalence of HCV, HBV and HIV infection among hemophilia patients. Hakim Res J. 2003;6(2):45-52. [Persian].
  14. Fransen van de Putte DE, Fischer K, Makris M, et al. Unfavourable cardiovascular disease risk profiles in a cohort of Dutch and British haemophilia patients. Thromb Haemost. 2013;109(1):16-23. doi: 10.1160/TH12-05-0332.
  15. Kulkarni R, Soucie JM, Evatt B. Renal disease among males with haemophilia. Haemophilia. 2003;9(6):703-710. doi: 10.1046/j.1351-8216.2003.00821.x.
  16. Klinge J, Auberger K, Auerswald G, Brackmann HH, Mauz- Korholz C, Kreuz W. Prevalence and outcome of intracranial haemorrhage in haemophiliacs--a survey of the paediatric group of the German Society of Thrombosis and Haemostasis (GTH). Eur J Pediatr. 1999;158 Suppl 3:S162-165. doi: 10.1007/PL00014346.
  17. Naderi M, Zarei T, Haghpanah S, Eshghi P, Miri-Moghaddam E, Karimi M. Intracranial hemorrhage pattern in the patients with factor XIII deficiency. Ann Hematol. 2014;93(4):693-697. doi: 10.1007/s00277-013-1918-7.
  18. Triemstra M, Rosendaal FR, Smit C, Van der Ploeg HM, Briet E. Mortality in patients with hemophilia. Changes in a Dutch population from 1986 to 1992 and 1973 to 1986. Ann Intern Med. 1995;123(11):823-827. doi:10.7326/0003-4819-123- 11-199512010-00002.
  19. Tagliaferri A, Rivolta GF, Iorio A, et al. Mortality and causes of death in Italian persons with haemophilia, 1990-2007. Haemophilia. 2010;16(3):437-446. doi:10.1111/j.1365- 2516.2009.02188.x.