Introduction: β-Thalassemia syndromes are among the most common monogenic disorders worldwide. Clinically, on the basis of the severity of the phenotype, β-thalassemias are classified into two groups: transfusion-dependent thalassemia (TDT) and non-transfusion-dependent thalassemia (NTDT). In the last few decades, considerable advances in understanding the pathophysiology of β-thalassemia have significantly improve d patient management, which has led to an increase in the life span of these subjects. However, new complications associated with aging are emerging, and β-thalassemias are becoming a growing concern for the health care systems.Areas covered: The present review focused on the age-related complications in adults with β-thalassemia. Among the cardiovascular diseases, which remain a major cause of morbidity, pulmonary hypertension and arrhythmias are exhibiting increased prevalence. Adrenal insufficiency and bone disease are emerging as endocrinological complications that require proper treatment. Moreover, age-related complications observed in the general population, including cancers and renal disease, should not be neglected.Expert opinion: The present study reviews the management of above-stated complications in adults with β-thalassemia based on the experience of a referral center. It is noteworthy that clinical trials in this context are limited, and the expert opinion offered in the present report stems mainly from direct clinical experience.
Management of age-associated medical complications in patients with β-thalassemia / I. Motta, M. Mancarella, A. Marcon, M. Vicenzi, M.D. Cappellini. - In: EXPERT REVIEW OF HEMATOLOGY. - ISSN 1747-4086. - 13:1(2019), pp. 85-94. [10.1080/17474086.2020.1686354]
Management of age-associated medical complications in patients with β-thalassemia
I. Motta;M. Mancarella;A. Marcon;M. Vicenzi;M.D. Cappellini
2019
Abstract
Introduction: β-Thalassemia syndromes are among the most common monogenic disorders worldwide. Clinically, on the basis of the severity of the phenotype, β-thalassemias are classified into two groups: transfusion-dependent thalassemia (TDT) and non-transfusion-dependent thalassemia (NTDT). In the last few decades, considerable advances in understanding the pathophysiology of β-thalassemia have significantly improve d patient management, which has led to an increase in the life span of these subjects. However, new complications associated with aging are emerging, and β-thalassemias are becoming a growing concern for the health care systems.Areas covered: The present review focused on the age-related complications in adults with β-thalassemia. Among the cardiovascular diseases, which remain a major cause of morbidity, pulmonary hypertension and arrhythmias are exhibiting increased prevalence. Adrenal insufficiency and bone disease are emerging as endocrinological complications that require proper treatment. Moreover, age-related complications observed in the general population, including cancers and renal disease, should not be neglected.Expert opinion: The present study reviews the management of above-stated complications in adults with β-thalassemia based on the experience of a referral center. It is noteworthy that clinical trials in this context are limited, and the expert opinion offered in the present report stems mainly from direct clinical experience.File | Dimensione | Formato | |
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