Uses and Limitations of Serum Ferritin, Magnetic Resonance Imaging T2 and T2 in the Diagnosis of Iron Overload and in the Ferrikinetics of Normalization of the Iron Stores in Thalassemia Using the International Committee on Chelation Deferiprone Deferoxamine Combination Protocol

• Published in: Hemoglobin Vol. 33; no. 5; pp. 312 - 322
• Main Authors: Kolnagou, Anita, Yazman, Dilek, Economides, Charalambos, Eracleous, Eleni, Kontoghiorghes, George J.
• Format: Journal Article
• Language: English
• Published: England Informa UK Ltd 01.10.2009; Taylor & Francis
• Subjects: Adolescent; Adult; Chelation Therapy - methods; Child; Deferiprone (L1); Deferoxamine (DFO); Deferoxamine - administration & dosage; Deferoxamine - therapeutic use; Diagnosis, Differential; Drug Therapy, Combination; Female; Ferrikinetics; Ferritins - blood; Humans; Iron - blood; Iron - metabolism; Iron Chelating Agents - administration & dosage; Iron Chelating Agents - therapeutic use; Iron Overload - diagnosis; Iron Overload - drug therapy; Liver - metabolism; Magnetic Resonance Imaging; Magnetic resonance imaging (MRI) T2 and T2diagnosis; Male; Middle Aged; Myocardium - metabolism; Pyridones - administration & dosage; Pyridones - therapeutic use; Serum ferritin; Siderophores - administration & dosage; Siderophores - therapeutic use; Thalassemia; Thalassemia - drug therapy; Young Adult
• ISSN: 0363-0269; 1532-432X
• DOI: 10.3109/03630260903213231

Excess cardiac iron deposition leads to congestive cardiac failure and accounts for more than 70% of deaths in thalassemia major patients. In three separate studies involving 145 thalassemia patients, serum ferritin and magnetic resonance imaging (MRI) relaxation times T2 and T2* have been compared for assessing iron load levels during chelation treatment. In two studies, variable levels of cardiac iron load have been detected by T2 and T2* in patients treated with deferoxamine (DFO), which, however, were unrelated to serum ferritin. In most cases, similar range levels from normal to severe cardiac iron load could be identified by both the T2 and T2* methods. However, in a few cases there were substantial differences in the levels detected between the two methods. In the third study, the ferrikinetics of the normalization of the iron stores during the International Committee on Chelation (ICOC) deferiprone (L1) DFO combination protocol was followed up using T2 and T2* and serum ferritin. Iron deposits were found not to be proportionally distributed between the liver and the heart or uniformly distributed within each organ. Iron mobilization in each patient varied and iron deposits in each organ were cleared at different rates. Despite some limitations, the application of the MRI relaxation times T2 and T2* offers the best diagnostic methods for iron overload estimations in most organs and especially the heart. These MRI methods and serum ferritin could also be used for the ferrikinetics of iron mobilization and removal during chelation therapy and the normalization of the iron stores during the ICOC L1 DFO combination protocol. There is a need to standardize the two MRI relaxation times T2 and T2* methods and identify the factors causing the differences between them.