Nutritional Assessment in Patients with Transfusion Dependent β Thalassemia

Abstract

Background: Transfusion dependent β thalassemia is a chronic inherited hemolytic anemia with defective hemoglobin β-chain.  Nutritional deficiencies associations are being recognized in recent research. Although it is not fully understood, it might be related to  increased nutrients needed, secondary hemochromatosis, endocrinopathies, and decreased Red Cells’ life span. The aim of this study is  to assess the nutritional deficiencies in Beta thalassemia major.

Methods: A cross-sectional study on fifty transfusion dependent β  thalassemia major patients ranging in age from 19 to 42 years old that were recruited from Benha Hematology Outpatient Clinic and  Internal Medicine Admission wards, Benha University Hospitals, Egypt from February 2022 to November 2022. Hematological Indices like  Hemoglobin, Platelets, and White Blood Cells were evaluated. Ferritin was tested in addition to Vitamin D 3 assay, Parathyroid hormone,  Hb A1c, thyroid stimulating hormone, and DEXA scan. Also, extensive nutritional laboratory tests such as serum total protein, albumin,  folate, calcium, Phosphorus, and Magnesium were investigated. In addition to height, weight, and routine laboratory evaluation,  comprehensive history was taken.

Results: Serum Vitamin D, folate and PTH were the most deficient in our studied patients representing  82 %, 28 %, and 20 % respectively. Also, bone mineral density was abnormal in 6 (12%) patients who had either osteopenia or osteoporosis. An increased prevalence of Type 2 Diabetes Mellitus (14%) was noted. No significant association between serum ferritin  level and nutritional deficiency was found.

Conclusions: Early assessment of nutritional deficiencies in Transfusion dependent β  thalassemia is a critical step in their evaluation. Because our adult thalassaemic patient showed a lesser prevalence of the underweight  category, BMI might be an inaccurate method for nutritional assessment. The vitamin D essay, serum calcium, Hb A1c, and DEXA scan should be the first screening investigations in the thalassemia patient’s evaluation.