BACKGROUND: Patients with β thalassemia intermedia can have substantial iron overload, irrespectively of their transfusion status, secondary to increased intestinal iron absorption. This study evaluates whether iron overload in patients with β thalassemia intermedia is associated with morbidity. DESIGN AND METHODS: This was a cross-sectional study of 168 patients with β thalassemia intermedia treated at two centers in Lebanon and Italy. Data on demographics, splenectomy status, transfusion status, and presence of co-morbidities were retrieved. Laboratory values of serum ferritin, fetal and total hemoglobin levels, as well as platelet and nucleated red blood cell counts were also obtained. Iron burden was determined directly by measuring liver iron concentration using magnetic resonance imaging. Patients were subdivided according to transfusion and splenectomy status into groups with phenotypes of different severity. RESULTS: The mean age of the patients was 35.2 ± 12.6 years and 42.9% of them were male. The mean liver iron concentration was 8.4 ± 6.7 mg Fe/g dry weight. On multivariate logistic regression analysis, after adjusting for age, gender, splenectomy status, transfusion status, and laboratory indices, an increase in 1 mg Fe/g dry weight liver iron concentration was independently and significantly associated with higher odds of thrombosis, pulmonary hypertension, hypothyroidism, osteoporosis, and hypogonadism. A liver iron concentration of at least 7 and at least 6 mg Fe/g dry weight were the best thresholds for discriminating the presence and absence of vascular and endocrine/bone morbidities, respectively (area under the receiver-operating characteristic curve: 0.72, P<0.001). Elevated liver iron concentration was associated with an increased rate of morbidity in patients with phenotypes of all severity, with a steeper increase in the rate of vascular morbidity being attributed to aging, and an earlier appearance of endocrine and bone disease. CONCLUSIONS: Elevated liver iron concentration in patients with β thalassemia intermedia is a marker of increased vascular, endocrine, and bone disease.
BACKGROUND:Patients with β thalassemia intermedia can have substantial iron overload, irrespectively of their transfusion status, secondary to increased intestinal iron absorption. This study evaluates whether iron overload in patients with β thalassemia intermedia is associated with morbidity. DESIGN AND METHODS: This was a cross-sectional study of 168 patients with β thalassemia intermedia treated at two centers in Lebanon and Italy. Data on demographics, splenectomy status, transfusion status, and presence of co-morbidities were retrieved. Laboratory values of serum ferritin, fetal and total hemoglobin levels, as well as platelet and nucleated red blood cell counts were also obtained. Iron burden was determined directly by measuring liver iron concentration using magnetic resonance imaging. Patients were subdivided according to transfusion and splenectomy status into groups with phenotypes of different severity. RESULTS: The mean age of the patients was 35.2 ± 12.6 years and 42.9% of them were male. The mean liver iron concentration was 8.4 ± 6.7 mg Fe/g dry weight. On multivariate logistic regression analysis, after adjusting for age, gender, splenectomy status, transfusion status, and laboratory indices, an increase in 1 mg Fe/g dry weight liver iron concentration was independently and significantly associated with higher odds of thrombosis, pulmonary hypertension, hypothyroidism, osteoporosis, and hypogonadism. A liver iron concentration of at least 7 and at least 6 mg Fe/g dry weight were the best thresholds for discriminating the presence and absence of vascular and endocrine/bone morbidities, respectively (area under the receiver-operating characteristic curve: 0.72, P<0.001). Elevated liver iron concentration was associated with an increased rate of morbidity in patients with phenotypes of all severity, with a steeper increase in the rate of vascular morbidity being attributed to aging, and an earlier appearance of endocrine and bone disease. CONCLUSIONS: Elevated liver iron concentration in patients with β thalassemia intermedia is a marker of increased vascular, endocrine, and bone disease.
Authors: Ellen B Fung; Paul R Harmatz; Phillip D K Lee; Meredith Milet; Rita Bellevue; Michael R Jeng; Karen A Kalinyak; Mark Hudes; Suruchi Bhatia; Elliott P Vichinsky Journal: Br J Haematol Date: 2006-10-10 Impact factor: 6.998
Authors: Angela O'Donnell; A Premawardhena; M Arambepola; S J Allen; T E A Peto; C A Fisher; D C Rees; Nancy F Olivieri; D J Weatherall Journal: Proc Natl Acad Sci U S A Date: 2007-05-17 Impact factor: 11.205
Authors: David M Nathan; John B Buse; Mayer B Davidson; Ele Ferrannini; Rury R Holman; Robert Sherwin; Bernard Zinman Journal: Diabetes Care Date: 2008-10-22 Impact factor: 17.152
Authors: Erik R Anderson; Matthew Taylor; Xiang Xue; Sadeesh K Ramakrishnan; Angelical Martin; Liwei Xie; Bryce X Bredell; Sara Gardenghi; Stefano Rivella; Yatrik M Shah Journal: Proc Natl Acad Sci U S A Date: 2013-11-26 Impact factor: 11.205
Authors: Antonella Meloni; Jon Detterich; Alessia Pepe; Paul Harmatz; Tom D Coates; John C Wood Journal: Blood Cells Mol Dis Date: 2014-11-24 Impact factor: 3.039
Authors: Claudia R Morris; Hae-Young Kim; John Wood; John B Porter; Elizabeth S Klings; Felicia L Trachtenberg; Nancy Sweeters; Nancy F Olivieri; Janet L Kwiatkowski; Lisa Virzi; Sylvia T Singer; Ali Taher; Ellis J Neufeld; Alexis A Thompson; Vandana Sachdev; Sandra Larkin; Jung H Suh; Frans A Kuypers; Elliott P Vichinsky Journal: Haematologica Date: 2013-04-12 Impact factor: 9.941
Authors: Pierre Brissot; Thibault Cavey; Martine Ropert; François Gaboriau; Olivier Loréal Journal: Environ Sci Pollut Res Int Date: 2016-09-15 Impact factor: 4.223