Diabetic Ketoacidosis Presenting in Major Thalassemia With Pancreatic Hemosiderosis: A Case Report

Introduction: Iron overload is a common complication of thalassemia, responsible for multiple organ damage, including pancreas. The prevalence of diabetes in thalassemia was 9.7–29%,(1) while the incidence of diabetic ketoacidosis (DKA) is only 0,3%.(2) Case Illustration: A 26 year-old male with thalassemia major (TM) admitted due to dyspnea, which later revealed as first episode of DKA. No prior history of hyperglycemia, but the patient has unexplained weight loss. He receives combination of chelators (deferiprone, desferroxamine, deferriprox) with ferritin level 8,387 μg/mL. Serum glucose was remarkably high (690 mg/dL), with ketosis (4.2 μg/dL) and metabolic acidosis (pH 7.2; bicarbonate 5.6 mEq/L). No single insult was identified, but recurrent ventricular tachycardia was documented. T2* MRI demonstrates severe cardiac and mild pancreatic hemosiderosis. Echocardiography shows reduced ejection fraction (25.6%), atrioventricular dilatation, and hypokinetism. Discussion: Impaired glucose metabolism in TM is initially mediated by insulin resistance, but later, the interplay between defect in insulin secretion and resistance determines the various clinical picture of diabetes in TM.(3) Several mechanisms have been linked: oxidative stress, induction of autoimmunity, and zinc deficiency; yet, current evidence remains inadequate.(4) The T2* MRI provides the most reliable tool to predict the development of glycemic dysregulation, whereas relying solely on ferritin could lead to imprecise measurement of iron deposits.(5) The presence of cardiac abnormality might responsible for the DKA and suggests the need of rescue chelation for this patient. Conclusion: DKA is a rare endocrine complications which can be prevented by routine T2* MRI screening and has wide spectrum of clinical characteristics.