Rapid Iron Loading in Heart and Liver in a Patient with Transfusion Dependent Thalassaemia after Brief Poor Compliance with Iron Chelation Therapy

Sultan Qaboos University Medical Journal

Shahina Daar¹  Vasili Berdoukas²  Saeed Ahmed³ 

[1] Department of Haematology, College of Medicine & Health Sciences, Sultan Qaboos University, Muscat, Oman;Department of Hematology/Oncology, Children’s Hospital of Los Angeles, USA;Department of Medicine, Sultan Qaboos University Hospital, Muscat, Oman;

关键词: thalassaemia major;    iron overload;    deferiprone;    deferoxamine;    chelation therapy;    magnetic resonance imaging;    case report;    oman;   

DOI  :  

来源: DOAJ

【 摘 要 】

Iron loading in patients with transfusion dependent thalassaemia is considered to occur primarily in the liver and, once the liver becomes saturated, other organs begin loading. We report here a splenectomised male patient who was treated for hepatitis C virus infection. Prior to starting antiviral therapy, his serum ferritin was maintained below 500 ng/ml with deferiprone monotherapy; cardiac T2* by magnetic resonance imaging was 48.8ms and hepatic T2* was 19.5ms. After twelve months of antiviral treatment during which time he was very poorly compliant with his deferoxamine chelation therapy, his ferritin had risen to 3820 ng/ml and cardiac and hepatic T2* findings were 12.7 ms and 14.5 ms respectively, indicating increased iron loading in both organs, but particularly in the heart. Fifteen months after recommencing combination chelation, his ferritin was 95 ng/ml and cardiac and hepatic T2* were 27.5 and 28.4ms respectively, indicating complete clearance of iron load in both organs. This case demonstrates that iron overload can develop rapidly and in some cases there is relatively rapid iron loading in the heart as compared to the liver.

【 授权许可】

Unknown