Treatment Status of Patients with Β-Thalassemia Major in Northern Iran: Thalassemia Registry System
Background: Electronic registry system of beta-thalassemia patients was run by Thalassemia Research Center (TRC) in 2017. The aim of the current study was presentation of therapeutic status in these patients at Mazandaran Province, Iran.
Methods: Therapeutic status variables including: Name of cities and hospitals, age and sex of patients, dependent and non-transfusion-dependent, starting age of the blood transfusion and iron-chelating agents, blood group and Rh, washed blood transfusion, abnormal antibody, transfusion reactions, mean hemoglobin during the last 3 months, type of iron chelators, iron chelators dosage, serum ferritin, and the use of hydroxyurea.
Results: Overall, 1831 patients were registered [891 male (48.7%)]. Mean age of patients was 30±9.7 yr. Average of hemoglobin levels for female and male were 9.1±5.1 and 9.4±6.3 gr/dl, respectively. Seventy-six percent of transfusion-dependent patients (1385) have received iso-group PRBC (packed red blood cells), after crossmatch. The most common blood group among patient was type O-positive (35.7%). Monotherapy with desferrioxamine was most type of used iron-chelating agent in these patients (47.2%). Mean of ferritin was 3300±7800 (ng/ml). Twenty-eight percent of patients (484) have received hydroxyurea; proportion of male and female was approximately equal. T2 weighted magnetic resonance imaging (MRIT2*) was measured in 62.2% of patients. Moderate and severe hepatosiderosis was 10.1% and 2.9%, respectively. Patients with moderate and severe cardiac siderosis were 11% and 5%, respectively.
Conclusion: Registry findings are valuable for treatment management and ensuring patients medications. It will also provide accessibility to various levels of patients’ information for health care managers and experts to help them make appropriate decisions.
2. Taher AT, Musallam KM, Karimi M et al (2010). Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the OPTIMAL CARE study. Blood,115(10):1886-92.
3. Rachmilewitz EA, Giardina PJ (2011). How I treat thalassemia. Blood,118(13):3479-88.
4. Taher AT, Musallam KM, Cappellini MD, Weatherall DJ (2011). Optimal management of β thalassaemia intermedia. Br J Haematol,152(5):512-23.
5. Taher AT, Musallam KM, Karimi M, Cappellini MD (2012). Contemporary approaches to treatment of beta-thalassemia intermedia. Blood Rev,26(1):S24-7.
6. Higgs DR, Engel JD, Stamatoyannopoulos G (2012). Thalassaemia. The Lancet,379(9813):373-83.
7. Karimi M, Cohan N, De Sanctis V et al (2014). Guidelines for diagnosis and management of Beta-thalassemia intermedia. Pediatr Hematol Oncol,31(7):583-96.
8. Azarkeivan A, Hashemieh M, Shirkavand A, Sheibani K (2016). Correlation between heart, liver and pancreas hemosiderosis measured by MRI T2* among thalassemia major patients from Iran. Arch Iran Med,19(2):96-100.
9. Moradi G, Ghaderi E (2013). Chronic disease program in Iran: Thalassemia control program. Chronic Dis J,1(2):98-106.
10. Miri M, Tabrizi Namini M et al (2013). Thalassemia in Iran in last twenty years: the carrier rates and the births trend. IJBC,6(1):11-7.
11. Taher A, Isma'eel H, Cappellini MD (2006). Thalassemia intermedia: revisited. Blood Cells Mol Dis,37(1):12-20.
12. Taher A, Isma’eel H, Mehio G et al (2006). Prevalence of thromboembolic events among 8,860 patients with thalassaemia major and intermedia in the Mediterranean area and Iran. Thromb Haemost,96(4):488-91.
13. Abolghasemi H, Amid A, Zeinali S et al (2007). Thalassemia in Iran: epidemiology, prevention, and management. J Pediatr Hematol Oncol,29(4):233-8.
14. Kosaryan M, Vahidshahi K, Karami H et al (2007). Survival of thalassemic patients referred to the Boo Ali Sina teaching hospital, Sari, Iran. Hemoglobin,31(4):453-62.
15. Rajaeefard A, Hajipour M, Tabatabaee HR et al (2015). Analysis of survival data in thalassemia patients in Shiraz, Iran. Epidemiol Health,37: e2015031.
16. Karami H, Kowsaryan M, Vahidshahi K et al (2010). Assessment of demographic, clinical and laboratory status of patients with thalassemia major and intermedia referred to thalassemia research center in Sari, Iran, during 2007-2009. Pajoohande,15(4):186-92.
17. Gharehbaghian A, Abolghasemi H, Namini MT (2008). Status of blood transfusion services in Iran. Asian J Transfus Sci,2(1):13-17.
18. Abolghasemi H, Maghsudlu M, Kafi-Abad SA, Cheraghali A (2009). Introduction to Iranian blood transfusion organization and blood safety in Iran. Iran J Public Health,38(1):82-7.
19. Cappellini M-D, Cohen A, Eleftheriou A, Piga A, Porter J, Taher A. Blood Transfusion Therapy in β-Thalassaemia Major. Guidelines for the Clinical Management of Thalassaemia [Internet] 2nd Revised edition 2008.
20. Kolnagou A, Natsiopoulos K, Kleanthous M et al (2013). Liver iron and serum ferritin levels are misleading for estimating cardiac, pancreatic, splenic and total body iron load in thalassemia patients: factors influencing the heterogenic distribution of excess storage iron in organs as identified by MRI T2. Toxicol Mech Methods,23(1):48-56.
21. Argyropoulou MI, Astrakas L (2007). MRI evaluation of tissue iron burden in patients with β-thalassaemia major. Pediatr Radiol,37(12):1191-1200.
22. Wood JC (2007). Magnetic resonance imaging measurement of iron overload. Curr Opin Hematol,14(3):183-90.
23. Kosaryan M, Zafari M, Alipur A, Hedayatizadeh-Omran A (2014). The effect and side effect of hydroxyurea therapy on patients with β-thalassemia: a systematic review to December 2012. Hemoglobin,38(4):262-71.
24. Kosaryan M, Karami H, Zafari M, Yaghobi N (2014). Report on patients with non transfusion-dependent β-thalassemia major being treated with hydroxyurea attending the Thalassemia Research Center, Sari, Mazandaran Province, Islamic Republic of Iran in 2013. Hemoglobin,38(2):115-8.
25. Kosaryan M, Vahidshahi K, Karami H, Ehteshami S (2009). Effect of hydroxyurea on thalassemia major and thalassemia intermedia in Iranian patients. Pak J Med Sci,25(1):74-8.
26. Mojtahedzadeh F, Kosaryan M, Mahdavi M-R, Akbari J (2006). The effect of folic acid supplementation in beta-thalassemia major: a randomized placebo-controlled clinical trial. Arch Iran Med,9(3):266-8.
|Issue||Vol 48 No 7 (2019)|
|Beta thalassemia major Intermedia thalassemia Deferoxamine Deferiprone Deferasirox|
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