Indian Journal of Medical Specialities

LETTER TO THE EDITOR
Year
: 2021  |  Volume : 12  |  Issue : 1  |  Page : 44--45

Dyslipidemia in adult thalassemia patients


Shubha Laxmi Margekar, Ritika Sud, Ramesh Aggarwal 
 Department of Medicine, Lady Hardinge Medical College and Associated Hospitals, New Delhi, India

Correspondence Address:
Dr. Shubha Laxmi Margekar
Room No. 1014, Department of Medicine, Lady Hardinge Medical College & SSKH, Panchkuin Road, New Delhi - 110 001
India




How to cite this article:
Margekar SL, Sud R, Aggarwal R. Dyslipidemia in adult thalassemia patients.Indian J Med Spec 2021;12:44-45


How to cite this URL:
Margekar SL, Sud R, Aggarwal R. Dyslipidemia in adult thalassemia patients. Indian J Med Spec [serial online] 2021 [cited 2022 Jan 18 ];12:44-45
Available from: http://www.ijms.in/text.asp?2021/12/1/44/306250


Full Text



Dear Editor,

Beta thalassemia is one of the most frequently seen autosomal recessive blood disorder. Over the past few decades, thalassemia has changed from fatal disease of early childhood to a chronic disease of adulthood. Regular blood transfusions and easy availability of iron chelation therapy have drastically improved the quality of life and increasing life expectancy into the third decade and beyond. Adult thalassemia patients have higher incidence of complications, which may be because of inadequate transfusions and/or chelation, transfusion-related complications such as viral infections, iron overload (hepatic, cardiac, and endocrine) and allosensitization, iron-chelator drug toxicities, osteoporosis and psychosocial issues.[1] Hence, awareness and knowledge are very important for physicians managing adult thalassemia individuals.

We read with interest the article on hypertriglyceridemia in thalassemia patient.[2] Dyslipidemia has been reported in various hematological disorders such as sickle cell disease, glucose-6-phosphate dehydrogenase deficiency, spherocytosis, aplastic anemia, myelodysplastic syndrome, and different types of beta thalassemia. Suggested mechanisms of pathogenesis[3],[4],[5] include:

Plasma dilution due to anemiaIncreased cholesterol uptake by macrophages and histiocytes (of the reticuloendothelial system) due to accelerated erythropoiesisIron overload leading to deranged liver function (decreased hepatic biosynthesis), resulting in altered lipoprotein patternCytokine release causing macrophage system activationHormonal disturbances secondary to iron overload of endocrine organ systemsRise in circulating triglyceride because of decreased extra hepatic lipolytic activity.

Frequent blood transfusion leads to iron accumulation and overload in several organs namely heart, liver, and endocrine organs, and initiates hepatic acute-phase response. Therefore, with advancing age, the greater cumulative blood transfusions contribute to iron overload of several organs. Age in itself has a significant positive correlation with lipid values resulting in hypercholesterolemia and high low-density lipoprotein (LDL) cholesterol levels except that for high-density lipoprotein (HDL) cholesterol levels.[6] Several lifestyle factors such as dietary habits, smoking, glucose intolerance, and raised blood pressure specially, as thalassemia patients grow into adulthood affect the relationship between serum lipid profile levels and atherosclerotic changes. A significant decrease in total cholesterol, HDL-cholesterol, LDL-cholesterol values, lipoprotein (a) and apolipoproteins A1 and B has been reported in thalassemia patients, although triglycerides are found to be elevated.[6] In 192 young Greek thalassemia patients (average age males 25 years and females 26 years), total serum cholesterol values >200 mg/dL were observed in 4% males and 2% females, while hypertriglyceridemia (>150 mg/dL) was observed in 11% males and 17% females.

Higher the ratio of total cholesterol to HDL-cholesterol, more is the risk of thrombotic and atherosclerotic complications. Hypocholesterolemia and hypertriglyceridemia have been reported among thalassemia children as well.[7] The risk for myocardial infarction is also higher if HDL cholesterol is low, even with normal level of total cholesterol. Cardiac complications are the leading cause of mortality in patients of thalassemia. Cardiac damage occurs primarily because of iron overload resulting in thalassemic cardiomyopathy. Arrhythmias or sudden death may occur without any signs of cardiac disease in patients of thalassemia. The serum triglyceride values correlate negatively with RBCs transfusion index and have positive correlation with serum ferritin levels in thalassemia patients.

To conclude, deranged lipid profile makes the thalassemia subjects more prone for atherogenic and thrombotic complications. The factors contributing to dyslipidemia are mainly iron overload and oxidative stress. A regular follow-up and serum lipid profile should be done in all adult thalassemia patients for early detection and timely intervention. These patients with altered lipid profile should be on dietary and lifestyle modification, and pharmacological management, as and when needed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Prakash A, Aggarwal R. Thalassemia major in adults: Short stature, hyperpigmentation, inadequate chelation, and transfusion-transmitted infections are key features. N Am J Med Sci 2012;4:141-4.
2Pandey S, Agrawal P, Rawat A, Gupta M, Khare P. Hypertriglyceridemia in a baby with thalassemia major. Indian J Med Spec 2020;11:157-60.
3Ragab SM, Safan MA, Sherif AS. Lipid profiles in β thalassemic children. Menoufia Med J 2014;27:66-72.
4Haghpanah S, Davani M, Samadi B, Ashrafi A, Karimi M. Serum lipid profiles in patients with beta-thalassemia major and intermedia in southern Iran. J Res Med Sci 2010;15:150-4.
5Nasir C, Rosdiana N, Lubis AD. Correlation between 25-hydroxyvitamin D and lipid profile among children with beta thalassemia major. Open Access Maced J Med Sci 2018;6:1790-4.
6Chrysohoou C, Panagiotakos DB, Pitsavos C, Kosma K, Barbetseas J, Karagiorga M, et al. Distribution of serum lipids and lipoproteins in patients with beta thalassaemia major; an epidemiological study in young adults from Greece. Lipids Health Dis 2004;3:3.
7Doshi S, Sutay NR. Study of serum lipid profile in beta-thalassemia major patients. JMSCR 2016;4:11613-21. Available from: http://jmscr.igmpublication.org/v4-i7/71%20jmscr.pdf. [Last accessed on 2020 Oct 18].