Main Article Content
Background: Endocrinopathies are well recognized serious complications among thalassemia patients with iron overload. The predicting serum ferritin level for endocrinopathies has not been determined.
Objective: The study aimed to evaluate the correlation between serum ferritin levels and endocrinopathies among thalassemia patients.
Materials and Methods: This study employed a cross-sectional design. All patients with thalassemia, age >18 years were enrolled between August 2011 and December 2012. Fasting blood sugar, thyroid and gonadal functions were evaluated. Serum ferritin, non–transferrin binding iron (NTBI) and labile plasma iron (LPI) were measured simultaneously.
Result: In all, 118 patients with thalassemia with a median age of 28 years (18-71) were enrolled. The majority of patients presented β-thalassemia/hemoglobin E (49.2%), followed by homozygous β-thalassemia (28.8%) and hemoglobin H disease (20.3%). Most patients (58.5%) underwent splenectomy while two thirds (62.7%) were transfusion dependent thalassemia (TDT). The mean spot and maximum ferritin levels were 2,379 μg/L (279-9,817) and 4,914 μg/L (279-37,656), respectively. The prevalence of diabetes mellitus, hypothyroidism, subclinical hypothyroidism and hypogonadism were 11.9%, 7%, 23.5% and 34.8%, respectively. NTBI correlated well with LPI, serum iron and transferrin saturation. The maximum ferritin level of > 3,000 μg/L was independently associated with diabetes [OR 9.94 (95%CI: 1.34-79.27), p = 0.004], hypothyroidism [OR 3.73 (95%CI: 1.51-9.20), p = 0.003] and hypogonadism [OR 3.54 (95%CI: 1.63-7.69), p = 0.001].
Conclusion: A high prevalence of endocrinopathies was found among adults with thalassemia. The maximum ferritin level of > 3,000 μg/L proved a major risk factor of developing diabetes, hypothyroidism and hypogonadism.
2. Origa R, Galanello R. Pathophysiology of beta thalassaemia.Pediatr Endocrinol Rev. 2011;8:263-70.
3. Ballas SK. Iron overload is a determinant of morbidity and mortality in adult patients with sickle cell disease. Semin Hematol. 2001;38(Suppl 1):30-6.
4. Hurrell R, Egli I. Iron bioavailability and dietary reference values. Am J Clin Nutr. 2010;91:1461-7.
5. Vannasaeng S, Ploybutr S, Visutkul P, Tandhanand S, Suwanik R, Wasi P. Endocrine function in thalassaemia. Clin Endocrinol. 1981;14:165-73.
6. Jaruratanasirikul S, Wongcharnchailert M, Laosombat V, Sangsupavanich P, Leetanaporn K. Thyroid function in beta-thalassemic children receiving hypertransfusions with suboptimal iron-chelating therapy. J Med Assoc Thai. 2007; 90:1798-802.
7. Jaruratanasirikul S, Chareonmuang R, Wongcharnchailert M, Laosombat V, Sangsupavanich P, Leetanaporn K. Prevalence of impaired glucose metabolism in beta-thalassemic children receiving hypertransfusions with a suboptimal dosage of iron chelating therapy. Eur J Pediatr. 2008;167:873-6.
8. Jaruratanasirikul S, Tanchotikul S, Wongcharnchailert M, Laosombat V, Sangsupavanich P, Leetanaporn K. A low dose adrenocortico tropin test (1 microg ACTH) for the evaluation of adrenal function in children with beta-thalassemia receiving hypertransfusion with suboptimal iron-chelating therapy. J Pediatr Endocrinol Metab. 2007;20:1183-8.
9. Kohgo Y, Ikuta K, Ohtake T, Torimoto Y, Kato J. Body iron metabolism and pathophysiology of iron overload. Int J Hematol 2008;88:7-15.
10. Hershko C, Graham G, Bates GW, Rachmilewitz EA. Non-specific serum iron in thalassemia: an abnormal serum iron fraction of potential toxicity. Br J Haematol. 1978;40:255-63.
11. Domenica Cappellini M, Tavazzi D, Duca L, Marelli S, Fiorelli G. Non-transferrin-bound iron, iron-related oxidative stress and lipid peroxidation in beta-thalassemia intermedia. Transfus Sci 2000;23:245-6.
12. Esposito BP, Breuer W, Sirankapracha P, Pootrakul P, Hershko C, Cabantchik ZI. Labile plasma iron in iron overload: redox activity and susceptibility to chelation. Blood. 2003;102:2670-7.
13. Pootrakul P, Breuer W, Sametband M, Sirankapracha P, Hershko C, Cabantchik ZI. Labile plasma iron (LPI) as an indicator of chelatable plasma redox activity in iron overloaded beta-thalassemia/Hb E patients treated with an oral chelator. Blood. 2004;104:1504-10.
14. Piga A, Longo F, Duca L, Roggero S, Vinciguerra T, Calabrese R, et al. High nontransferrin bound iron levels and heart disease in thalassemia major. Am J Hematol. 2009;84:29-33.
15. Singer ST, Vichinsky EP, Gildengorin G, van Disseldorp J, Rosen M, Cedars M. Reproductive capacity in iron overload women with thalassemia major. Blood. 2011;118:2878-81.
16. Greenberg PL, Gordeuk V, Issaragrisil S, Siritanaratkul N, Fucharoen S, Ribeiro RC. Major hematologic diseases in the developing world-new aspects of diagnosis and management of thalassemia, malaria anemia, and acute leukemia. Hematology Am Soc Hematol Educ Program. 2001:479-98.
17. Pansatiankul B, Saisorn S. A community-based thalassemia prevention and control model in northern Thailand. J Med Assoc Thai. 2003;86:S576-82.
18. Panich V, Pornpatkul M, Sriroongrueng W. The problem of thalassemia in Thailand. Southeast Asian J Trop Med Public Health. 1992;23:1-6.
19. Siah CW, Ombiga J, Adams LA, Trinder D, Olynyk JK. Normal iron metabolism and the pathophysiology of overload disorders. Clin Biochem Rev. 2006;27:5-16.
20. Toumba M, Sergis A, Kanaris C, Skordis N. Endocrine complications in patients with Thalassaemia Major. Pediatr Endocrinol Rev. 2007;5:646-8.
21. Ong CK, Lim SL, Tan WC, Ong EE, Goh AS. Endocrine complications in transfusion dependent thalassaemia in Penang Hospital.Med J Malaysia. 2008;63:109-12.
22. Shamshirsaz AA, Bekheirnia MR, Kamgar M, Pourzahedgilani N, Bouzari N, Habibzadeh M, et al. Metabolic and endocrinologic complications in beta-thalassemia major: a multicenter study in Tehran. BMC Endocr Disord. 2003;12;3:4.
23. Vogiatzi MG, Macklin EA, Trachtenberg FL, Fung EB, Cheung AM, Vichinsky E, et al. Differences in the prevalence of growth, endocrine and vitamin D abnormalities among the various thalassaemia syndromes in North America. Br J Haematol. 2009;146:546-56.
24. De Sanctis V, Eleftheriou A, Malaventura C. Prevalence of endocrine complications and short stature in patients with thalassaemia major: a multicenter study by the Thalassaemia International Federation (TIF). Pediatr Endocrinol Rev. 2004;2:249-55.
25. Gamberini MR, De Sanctis V, Gilli G. Hypogonadism, diabetes mellitus, hypothyroidism, hypoparathyroidism: incidence and prevalence related to iron overload and chelation therapy in patients with thalassaemia major followed from 1980 to 2007 in the Ferrara Centre. Pediatr Endocrinol Rev. 2008;6:158-69.
26. Najafipour F, Aliasgarzadeh A, Aghamohamadzadeh N, Bahrami A, Mobasri M, Niafar M, et al. A cross-sectional study of metabolic and endocrine complications in beta-thalassemia major. Ann Saudi Med. 2008;28:361-6.
27. Mehrvar A, Azarkeivan A, Faranoush M, Mehrvar N, Saberinedjad J, Ghorbani R, et al. Endocrinopathies in patients with transfusion-dependent beta-thalassemia. Pediatr Hematol Oncol. 2008;25:187-94.
28. Malik SA, Syed S, Ahmed N. Frequency of hypothyroidism in patients of beta-thalassaemia. J Pak Med Assoc. 2010;60:17-20.
29. De Sanctis V, De Sanctis E, Ricchieri P, Gubellini E, Gilli G, Gamberini MR. Mild subclinical hypothyroidism in thalassaemia major: prevalence, multigated radionuclide test, clinical and laboratory long-term follow-up study. Pediatr Endocrinol Rev.2008;6:174-80.
30. Jain M, Sinha RS, Chellani H, Anand NK. Assessment of thyroid functions and its role in body growth in thalassemia major. Indian Pediatr. 1995;32:213-9.
31. Filosa A, Di Maio S, Aloj G, Acampora C. Longitudinal study on thyroid function in patients with thalassemia major. J Pediatr Endocrinol Metab. 2006;19:1397-404.