Endocrine Gland Abnormalities in Thalassemia Major: Λ Brief Review

Abstract

Thalassemia major (beta-thalassemia) affects a significant segment of the population in certain areas of the world. Alterations in migration patterns have changed the geographic distribution of this disease and made it a worldwide health problem. Over the course of the past 2-3 decades hypertransfusion therapy has significantly increased the life expectancy, and improved the quality of life of these patients. At the same time there has been an increase in the frequency of complications of this therapy caused by iron overload. Endocrine gland abnormalities contributed little to the morbidity or mortality of beta-thalassemia in the past. As a result of hypertransfusion therapy and increased longevity, however, endocrinopathies have become more common and contribute significantly to morbidity in these patients. In this article we briefly review the current understanding of endocrine gland abnormalities, primarily caused by iron overload, in patients with thalassemia major.

Associated Complications In Beta Thalassemia Patients

“…Endocrine complications include diabetes mellitus, hypothyroidism, hypoparathyroidism, hypogonadism and delayed puberty. These complications have contributed little to morbidity and mortality in the past; however as a result of increased longevity, these have become more common and contribute significantly to the morbidity in these patients [17]. Endocrine complications along with osteoporosis, trace elements deficiency and other metabolic disturbances also lead to growth failure and short stature [9,10].…”

Prevalence of Hypoparathyroidism (HPT) in Beta Thalassemia Major

“…Despite a significant increase in the lifespan of these patients, many endocrine abnormalities such as hypogonadism, diabetes mellitus, hypothyroidism and HPT develop due to an iron overload [3]. Even though HPT is thought to be a rare complication, it may cause various neurological manifestations such as tetany, seizures, carpopedal spasms, and paresthesia [4,5]. In different studies which have been performed, the incidence of hypoparathyroid dysfunction varies from 0% up to almost 22.5% of patients [6].…”

Prevalence of impaired glucose metabolism in β-thalassemic children receiving hypertransfusions with a suboptimal dosage of iron-chelating therapy

“…Diabetes mellitus (DM) is a well-documented endocrine complication of transfusion-dependent β-thalassemic patients [18,22,25]. It is a relatively infrequent condition during the first decade of life, but progressively increases in frequency with increasing age, attaining its zenith between ages 16 and 20 years.…”