Skip to main content
Log in

Plasma lipids and lipoproteins in children and young adults with major β-thalassemia from western Iran: influence of genotype

  • Published:
Molecular Biology Reports Aims and scope Submit manuscript

Abstract

To determine the plasma lipid and lipoprotein profiles and their possible association with the type of β-thalassemia mutation we studied 103 major β-thalassemia patients including 71 children and 32 young adults compared to 102 healthy subjects consisted of 90 children and 12 young healthy adults. The plasma lipid and lipoprotein levels were measured by conventional methods. Considering all of the patients the levels of total cholesterol (TC), LDL-cholesterol (LDL-C), and HDL-cholesterol (HDL-C) were significantly lower compared to controls. However, the level of TG was significantly higher in cases than controls. Comparing thalassemic patients homozygous for a β0 type of mutation with those homozygous for a β+ type of mutation (IVSI.110 G:A) indicated that the levels of LDL-C, TC were significantly increased and TG concentration tended to be higher in the latter patients. In conclusion, our study indicates that hemolytic stress results in hypocholesterolemia in major β-thalassemia patients and the presence of more severe genotype in patients is correlated with more reduction in TG, TC, and LDL-C levels.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Chrysohoou C, Panagiotakos DB, Pitsavos C, Kosma K, Barbetseas J, Karagiorga M, Ladis I, Stefanadis C (2004) Distribution of serum lipids and lipoproteins in patients with beta thalassemia major: an epidemiological study in young adults from Greece. Lipid Health Dis 3:3

    Article  Google Scholar 

  2. Ricchi P, Ammirabile M, Spasiano A, Costantini S, Di Matola T, Cinque P, Pagano L, Prossomariti L (2009) Hypocholesterolemia in adult patients with thalassemia: a link with the severity of genotype in thalassemia intermedia patients. European J Haematol 82:219–222

    Article  Google Scholar 

  3. Amendola G, Danise P, Todisco N, D’urzo G, Di Palma A, Di Concilio R (2007) Lipid profile in β-thalassemia intermedia patients: correlation with erythroid bone marrow activity. Int J Lab Haematol 29:172–176

    Article  CAS  Google Scholar 

  4. Rahimi Z, Merat A, Haghshenass M, Madani H, Rezaei M, Nagel RL (2006) Plasma lipids in Iranians with sickle cell disease: hypocholesterolemia in sickle cell anemia and increase of HDL-cholesterol in sickle cell trait. Clin Chim Acta. 365:217–220

    Article  PubMed  CAS  Google Scholar 

  5. Rahimi Z, Vaisi-Raygani A, Pourmotabbed T (2010) Association between apolipoprotein ε4 allele, Factor V Leiden, and plasma lipid and lipoprotein Levels with sickle cell disease in Southern Iran. Mol Biol Rep (in press)

  6. Rahimi Z, Vaisi Raygani A, Merat A, Haghshenass M, Gerard N, Nagel RL, Krishnamoorthy R (2006) Thalassemic mutations in Southern Iran. Ir J Med Sci 31:70–73

    Google Scholar 

  7. Rahimi Z, Muniz A, Parsian A (2010) Detection of responsible mutations for beta thalassemia in the Kermanshah Province of Iran using PCR-based techniques. Mol Biol Rep 37:149–154

    Article  PubMed  CAS  Google Scholar 

  8. Rahimi Z, Muniz A, Mozafari H (2010) Abnormal Hemoglobins among Kurdish population of Western Iran: hematological and molecular features. Mol Biol Rep 37:51–57

    Article  PubMed  CAS  Google Scholar 

  9. Nemati H, Rahimi Z, Bahrami G (2010) The Xmn1 polymorphic site 5′ to the Gγ gene and its correlation to the Gγ:Aγ ratio, age at first blood transfusion and clinical features in β-thalassemia patients from Western Iran. Mol Biol Rep 37:159–164

    Article  PubMed  CAS  Google Scholar 

  10. Betke K, Marti HR, Schlict I (1959) Estimation of small percentages of fetal haemoglobin. Nature 184:1877–1878

    Article  PubMed  CAS  Google Scholar 

  11. Fairbanks VF, Klee GG (1994) Biochemical aspects of hematology. In: Burtis CA, Ashwood ER (eds) Tietz text book of clinical chemistry. W.B. Saunders, Philadelphia, pp 2041–2042

    Google Scholar 

  12. Old JM, Higgs DR (1983) Gene analysis. In: Weatherall DJ (ed) Methods in hemotology. Vol:6 The thalassemias. Livingstone Edinburgh pp 74–101

  13. Old JM (1996) Hemoglobinopathies. In: Elles R R (ed) Methods in molecular medicine: molecular diagnosis of genetic disease. Humana press Inc, Totowa, pp 169–183

    Chapter  Google Scholar 

  14. Rahim F, Kaikhaei B, Zandian K, Hoseini A (2008) Co-inheritance of alpha and beta-thalassemia in Khuzestan Province, Iran. Hematology 13:59–64

    Article  PubMed  CAS  Google Scholar 

  15. Abolghasemi H, Amid A, Zeinali S, Radfar MH, Eshghi P, Rahiminejad MS, Ehsani MA, Najmabadi H, Akbari MT, Afrasiabi A, Akhavan-Niaki H, Hoorfar H (2007) Thalassemia in Iran: epidemiology, prevention, and management. J Pediatr Hematol Oncol 29:233–238

    Article  PubMed  Google Scholar 

  16. Papanastasiou DA, Siorokou T, Haliotis FA (1996) Beta-Thalassaemia and factors affecting the metabolism of lipids and lipoproteins. Haematologica 27:143–153

    CAS  Google Scholar 

  17. Al-Quobaili FA, Abou Asali IE (2004) Serum levels of lipids and lipoproteins in Syrian patients with beta-thalassemia major. Saudi Med J 25:871–875

    PubMed  Google Scholar 

  18. Goldfarb AW, Rachmilewitz EA, Eisenberg S (1991) Abnormal low and high density lipoproteins in homozygous beta-thalassaemia. Br J Haematol 79:481–486

    Article  PubMed  CAS  Google Scholar 

  19. Maioli M, Cuccuru GB, Pranzetti P, Pacifico A, Cherchi GM (1984) Plasma lipids and lipoproteins pattern in beta-thalassemia major. Acta Haematol 71:106–110

    Article  PubMed  CAS  Google Scholar 

  20. Shores J, Peterson J, VanderJagt D, Glew RH (2003) Reduced cholesterol levels in African-American adults with sickle cell disease. J Natl Med Assoc 95:813–817

    PubMed  Google Scholar 

  21. Ghanizadeh A, Khajavian S, Ashkani H (2006) Prevalence of psychiatric disorders, depression, and suicidal behavior in child and adolescent with thalassemia major. J Pediatr Hematol Oncol 28:781–784

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Zohreh Rahimi.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Madani, H., Rahimi, Z., Manavi-Shad, M. et al. Plasma lipids and lipoproteins in children and young adults with major β-thalassemia from western Iran: influence of genotype. Mol Biol Rep 38, 2573–2578 (2011). https://doi.org/10.1007/s11033-010-0397-3

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11033-010-0397-3

Keywords

Navigation