Recovery of Bi-ventricular Dysfunction in Patient with Secondary Hemochromatosis with Multi-transfusions for Aplastic Anemia after Unrelated Allogeneic Peripheral Blood Stem Cell Transplantation

Abstract

Case report A 37-year-old male was referred to our institution for evaluation of heart failure in May 2017. He had been diagnosed to moderate aplastic anemia and had been treated with Anti-thymocyte globulin (ATG) chemotherapy and cyclosporine twice. He had been transfused with red blood cells and platelets weekly. Patient was suffered to dyspnea, orthopnea and epigastric pain and clinical examination showed tachycardia, rales, cardiomegaly on the chest x-ray, and ectopic atrial tachycardia with 108 bpm/min of heart rate and left atrial enlargement on the electrocardiogram. Echocardiography showed dilatation of all cardiac chambers (left ventricular end diastolic diameter (LVEDD) = 66 mm and indexed left atrial volume (LAVI) = 41ml/m2) with severe left ventricular (LV) dysfunction (left ventricular ejection fraction (LVEF) = 22%). Heart failure with reduced ejection fraction due to secondary hemochromatosis with multiple transfusions was thus diagnosed after consultation for hematology. Heart failure medications including furosemide, spironolactone, nebivolol, candesartan were started. Deferasirox was administered for hemochromatosis. We switched candesartan to Sacubitril/valsartan 1 month after first visit because of heart failure aggravation. Coronary angiography showed no significant stenosis and inserted implantable cardioverter defibrillator (ICD) due to refractory heart failure in August 2017. After ICD insertion, patient had several event of ventricular tachycardia (VT) and ventricular fibrillation (VF) with ICD appropriate shock. We added amiodarone to prevent further arrhythmic events. Three month after ICD insertion, he admitted to our hospital to undergo hematopoietic stem cell transplantation. Due to this severe cardiac dysfunction, he was treated using a myeloablative conditioning regimen with ATG followed by unrelated allogeneic peripheral blood stem cell transplantation (PBSCT) in November 2017. One month after PBSCT, echocardiography showed a dramatic improvement of LV systolic function (LVEF 25% -> 56%) and decreased cardiac chamber size (LVEDD = 65 -> 60 mm, LAVI = 33 -> 27ml/m2) and disappeared pericardial effusion. The patient's heart failure symptoms were almost resolved with medications of Sacubitril/valsartan, bisoprolol, and torsemide. Serum ferritin level (baseline 8803.6 ng/ml) was decreased to 4611.9 ng/ml after PBSCT. Here, we report a case of the patient who developed bi-ventricular dysfunction with secondary hemochromatosis due to multi-transfusions for aplastic anemia returned to normal cardiac function after successful allogeneic PBSCT with heart failure treatments. This case indicates that bi-ventricular dysfunction with secondary hemochromatosis due to multi-transfusions for aplastic anemia could be recovered after optimal medical treatment and PBSCT.