cardiovascular-agents and beta-Thalassemia

A 23-year-old male patient, who suffers from beta-thalassemia major, came to us for an endocrine-metabolic evaluation. Medical history showed a diagnosis of heart disease with heart failure since the age of 16, type 1 diabetes mellitus diagnosed at the age of 18, treated with an intensive insulin therapy with a poor glycometabolic control. Patient performed regular blood transfusions and iron chelation with deferasirox. An echocardiogram revealed an enlarged left ventricle. Patient had undergone a comprehensive study of buoyancy both basal and hormone-stimulated and it was therefore carried out a diagnosis of GH deficiency and hypogonadotropic hypogonadism. A recombinant GH replacement therapy was then prescribed. After six months of therapy, the patient reported a net improvement of asthenic symptoms. Physical examination showed a reduction in abdominal adiposity in waist and an increase of 5 cm in stature. Laboratory tests showed an amelioration of glycometabolic control, such as to justify a reduction in daily insulin dose. The stature observed was thought appropriate to begin the administration of testosterone. Moreover, the cardiological framework showed a reduction of left ventricular dilatation, good ventricular motility, global minimum persistent tricuspid but not mitral regurgitation and no alteration on ECG.

Topics: Asthenia; beta-Thalassemia; Blood Transfusion; Cardiovascular Agents; Chelation Therapy; Combined Modality Therapy; Diabetes Mellitus, Type 1; Dwarfism; Growth Hormone; Heart Failure; Human Growth Hormone; Humans; Hypogonadism; Insulin; Iron Chelating Agents; Iron Overload; Male; Mitral Valve Insufficiency; Testosterone; Tricuspid Valve Insufficiency; Young Adult

Cardiac complications caused by iron deposition are major causes of death in patients with beta-thalassemia major. Deferiprone (L1) was found to have greater efficacy at depleting myocardial iron than desferrioxamine (DFX). Furthermore, combined therapy with L1 and DFX produced an additive or synergistic iron chelating effect. We report the successful treatment of severe heart failure in two patients with beta-thalassemia major with the combined therapy. Magnetic resonance images showed a marked recovery of signal intensity in the heart, indicating a significant reduction of iron load in the heart. No significant adverse effects were noted. Therefore, combined therapy with L1 and DFX should be considered in patients with beta-thalassemia major and cardiac complications.

Topics: Adult; beta-Thalassemia; Cardiovascular Agents; Deferiprone; Deferoxamine; Diabetes Complications; Drug Therapy, Combination; Erythrocyte Transfusion; Female; Heart Failure; Hepatitis C, Chronic; Humans; Hypogonadism; Iron Chelating Agents; Iron Overload; Magnetic Resonance Imaging; Osteoporosis; Pyridones; Splenectomy; Transfusion Reaction; Tricuspid Valve Insufficiency