Case report Reversal of heart failure in thalassemia major by combined chelation therapy: a case report Case report The thalassemia major (MT) patient (28 yr,70 kg) was receiving desferrioxamine (DFO, Desferal Ò ; Novartis Pharma, Basel, Switzerland) 45 mg/kg/d s.c., with a poor compliance to the therapy. Mean Hb was 10 g/dL and mean ferritin level was 4500 lg/L with a maximum value 6200 lg/L. He was euthyroid, but hypogonadic, treated by 40 mg of testosterone p.o. daily. When admitted, he was orthopnoic, with extended pitting ankle edemas, and abdominal distension. On lung ausciltation, moist rales were heard bilaterally along with decreased bronchial breath sounds and a fourth cardiac sound was present. The jugular veins were palpable and dilated, while the clinical examination of the abdomen revealed a dilated abdomen with diffuse tenderness, ascites and liver enlargement. The arterial pressure was 90/60 mmHg. The chest roentgenogram revealed cardiomegaly (increased cardiothoracic ratio) and right lung pleural effu- sion. Hepatomegaly was confirmed by echo study, while liver enzymes were indicative of liver and cardiac dysfunction. The tripplex evaluation revealed biventricular dilation, left atrial dilatation, reduced contractility (Table 1), reduced right ven- tricular systolic function, pericardial effusion, and mitral regurgitation. Virological studies for Cox- sackie virus, CMV, influenza, and EBV were negative for recent infection and along with the patient’s clinical course excluded the possibility of myocarditis. Intensive iron chelation was initiated with DFO 50 mg/kg/d i.v. for 8–10 h daily/5 d a week and deferiprone (DFP, Ferriprox Ò ; Apotex Europe Ltd, Berkshire, England) 65 mg/kg/d. He was also given i.v. 500 mg dobutamine (1 mL/min) for 2 d/month during the first 6 months – in order to reduce afterload and gain time – furosemide (80 mg), enalapril (20 mg), and spironolactone (50 mg). He followed a total salt-free diet, full of dairy products. His body weight and the urine output were monitored closely. He underwent frequent pleural effusion aspirations for the first trimester. During therapy, hematological evalua- tion included hemoglobin, WBC count, absolute neutrophil count, and platelet count measurements every 10 d. Serum ferritin levels, liver, and renal function were controlled monthly. At the end of the first trimester, iron excretion during combination therapy was controlled and compared with the one during single DFO treatment, measuring urinary Tsironi M, Deftereos S, Andriopoulos P, Farmakis D, Meletis J, Aessopos A. Reversal of heart failure in thalassemia major by combined chelation therapy: a case report. Eur J Haematol 2005: 74: 84–85. Ó Blackwell Munksgaard 2005. Abstract: In patients with thalassemia major (TM) who are non-com- pliant with long-term desferrioxamine (DFO) chelation, survival is limited mainly because of cardiac complications of transfusional hemosiderosis. Combined chelation therapy with DFO and deferiprone has maximized the efficacy of the therapy and reduced cardiological complications. The aim of this report is to present the results of this combination in a desperate case of heart failure. M. Tsironi, S. Deftereos, P. Andriopoulos, D. Farmakis, J. Meletis, A. Aessopos First Department of Internal Medicine, University of Athens, Athens, Greece Key words: heart failure; thalassemia; chelation Correspondence: M. Tsironi, First Department of Internal Medicine, University of Athens, Athens, Greece Tel: +003-06932483226 Fax: +003-02731081140 e-mail: gpoyl@otenet.gr Accepted for publication 14 August 2004 Eur J Haematol 2005: 74: 84–85 All rights reserved Copyright Ó Blackwell Munksgaard 2005 EUROPEAN JOURNAL OF HAEMATOLOGY 84