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Elevated liver iron concentration is a marker of increased morbidity in patients with {beta} thalassemia intermedia.
MedLine Citation:
PMID:  21791471     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Background. Patients with β thalassemia intermedia can have substantial iron overload, irrespective of 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 β thalassemia intermedia patients treated at two centers in Lebanon and Italy. Data on demographics, splenectomy status, transfusion status, and presence of 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. Direct determination of iron burden was performed by liver iron concentration measurement using magnetic resonance imaging. Patients were subdivided to phenotype severity groups according to transfusion and splenectomy status. Results. The mean age was 35.2+/-12.6 years with 42.9% of patients being males. 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, a 1-mg Fe/g dry weight increase in liver iron concentration was independently and significantly associated with higher odds of thrombosis, pulmonary hypertension, hypothyroidism, osteoporosis, and hypogonadism. A liver iron concentration of ≥7 and ≥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 all phenotype severity groups, a steeper increase in the rate of vascular morbidity 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:
Khaled M Musallam; Maria Domenica Cappellini; John C Wood; Irene Motta; Giovanna Graziadei; Hani Tamim; Ali T Taher
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-7-26
Journal Detail:
Title:  Haematologica     Volume:  -     ISSN:  1592-8721     ISO Abbreviation:  -     Publication Date:  2011 Jul 
Date Detail:
Created Date:  2011-7-27     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0417435     Medline TA:  Haematologica     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Beirut, Lebanon;
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