Nutritional Deficiencies Are Common in Patients with Transfusion-Dependent Thalassemia and Associated with Iron Overload

Abstract

Patients with thalassemia are frequently deficient in key micronutrients. Attempts to correct these inadequacies through nutritional supplementation have been met with some success, although disparities between intake and circulating levels continue to be observed. This study employed a convenience sample of 41 well-nourished transfusion dependent patients with thalassemia to identify possible mechanisms behind nutritional deficiencies. Each subject completed a Block 2005© Food Frequency Questionnaire (FFQ), through which macro and micronutrient intake was quantified. Fasting blood was drawn to assess vitamins A, C, D, E, copper, selenium, zinc and hematologic parameters. Dietary intake was found to be inadequate compared to Institute of Medicine (IOM) recommendations for many of the fat-soluble vitamins, as well as calcium and zinc. Circulating deficiencies of vitamins C, D, copper, zinc and γ tocopherol were also present in over 20% of patients. Many individuals who consumed an adequate dietary intake had deficient levels of circulating nutrients, which suggest alternative etiologies of nutrient excretion or loss, in addition to higher micronutrient requirements. Liver iron concentration displayed a significant negative relationship with vitamins C (r=−0.62, p<0.001), E (r=−0.37, p=0.03), and zinc (r=−0.35, p=0.037), indicating that in iron-overloaded patients, these nutrients are either endogenously consumed at higher rates or sequestered within the liver, resulting in a functional nutrient deficiency. While this study identified hepatic iron overload to be a significant cause of nutritional deficits commonly observed in patients with thalassemia, multiple etiologies are simultaneously responsible. In response to these findings, nutritional status should be monitored regularly in at-risk patients with thalassemia, and prophylactically addressed with supplementation or aggressive chelation to avoid associated co-morbidities.

Moderate to severe liver siderosis and raised AST are independent risk factors for vitamin D insufficiency in β-thalassemia patients

“…Elevated serum iron levels ensues from blood transfusions and hyperabsorption of dietary iron by the gastrointestinal tract—especially in NTDT alongside reduced hepcidin as the hepatic peptide hormone regulating iron homeostasis 40 —are conducive to the iron siderosis in several organs such as tissue, cardiac, liver and also endocrine system glands, which harm their natural physiology. Subsequently, endocrinopathy and Vit-D deficiency as the adverse consequences resulting from superfluous iron burden can occur in patients with TDT 41 , 42 .…”

A Retrospective Long-Term Study on Age at Menarche and Menstrual Characteristics in 85 Young Women With Transfusion-Dependent Β-Thalassemia (Tdt) Born Between 1965 and 1995

“…28 The nutritional history of our patients did not document relevant deficiencies; however, we cannot fully exclude a reduced intake of macro and micronutrients. Goldberg et al 29 have reported that many TDT patients who consumed an adequate dietary intake had deficient levels of circulating nutrients. Liver iron concentration in TDT patients displayed a significant negative relationship with vitamins C, E, and zinc, indicating that in iron-overloaded patients, these nutrients are either endogenously consumed at higher rates or sequestered within the liver resulting in a functional nutrient deficiency.…”

Diagnosis and treatment of post-transfusion iron overload in pediatric hematology: literature review

“…Деферазирокс одобрен Управлением по контролю качества пищевых продуктов и лекарственных средств США (FDA) для использования у пациентов с перегрузкой железом в 2005 г. В исследованиях у политрансфузированных пациентов с МДС деферазирокс показал достоверное снижение уровня ферритина сыворотки крови [14]. В исследовании II фазы также показано, что деферазирокс достоверно снижает накопление железа в печени у политрансфузированных пациентов с МДС [21].…”