title: MRI guided iron assessment and oral chelator use improve iron status in thalassemia major patients.
abstract: Oral iron chelators and magnetic resonance imaging (MRI) assessment of heart and liver iron burden have become widely available since the mid 2000s, allowing for improved patient compliance with chelation and noninvasive monitoring of iron levels for titration of therapy. We evaluated the impact of these changes in our center for patients with thalassemia major and transfusional iron overload. This single center, retrospective observational study covered the period from 2005 through 2012. Liver iron content (LIC) was estimated both by a T2* method and by R2 (Ferriscan® ) technique. Cardiac iron was assessed as cT2*. Forty-two patients (55% male) with transfused thalassemia and at least two MRIs were included (median age at first MRI, 17.5 y). Over a mean follow-up period of 5.2 ± 1.9 y, 190 MRIs were performed (median 4.5 per patient). Comparing baseline to last MRI, 63% of patients remained within target ranges for cT2* and LIC, and 13% improved from high values to the target range. Both the median LIC and cT2* (cR2* = 1000/cT2*) status improved over time: LIC 7.3 to 4.5 mg/g dry weight, P = 0.0004; cR2* 33.4 to 28.3 Hz, P = 0.01. Individual responses varied widely. Two patients died of heart failure during the study period. Annual MRI iron assessments and availability of oral chelators both facilitate changes in chelation dose and strategies to optimize care.
authors.affiliation: Division of Hematology/Oncology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts.
authors.forename: Diana X
authors.initials: DX
authors.lastname: Nichols-Vinueza
authors.forename: Matthew T
authors.initials: MT
authors.lastname: White
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Powell
authors.forename: Puja
authors.initials: P
authors.lastname: Banka
authors.forename: Ellis J
authors.initials: EJ
authors.lastname: Neufeld
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D006801:Humans; D007501:Iron / Q000032:analysis* / Q000378:metabolism; D007502:Iron Chelating Agents / Q000008:administration & dosage*; D019190:Iron Overload / Q000188:drug therapy* / Q000378:metabolism* / Q000473:pathology; D008099:Liver / Q000378:metabolism / Q000473:pathology; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D055118:Medication Adherence; D009206:Myocardium / Q000378:metabolism / Q000473:pathology; D012189:Retrospective Studies; D055815:Young Adult; D017086:beta-Thalassemia / Q000188:drug therapy* / Q000378:metabolism*
chemical_list: D007502:Iron Chelating Agents; D007501:Iron