Document Detail


Iron overload in non-transfusion-dependent thalassemia: a clinical perspective.
MedLine Citation:
PMID:  22631036     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Iron overload due to increased intestinal iron absorption represents an important clinical problem in patients with non-transfusion-dependent thalassemia (NTDT), particularly as they advance in age. Current models for iron metabolism in patients with beta (β)-thalassemia intermedia (TI) suggest that suppression of serum hepcidin results in increased iron absorption and release of iron from the reticuloendothelial system, leading to depletion of macrophage iron, relatively low levels of serum ferritin, and liver iron loading. The clinical consequences of iron overload in patients with NTDT are multifactorial and include endocrinopathy, bone disease, thromboembolism, pulmonary hypertension, cerebrovascular and neuronal damage, liver fibrosis or cirrhosis, and increased risk of hepatocellular carcinoma. Although serum ferritin levels correlate with liver iron concentration (LIC), they underestimate iron load in these patients compared with transfusion-dependent patients with equivalent LIC. Therefore, direct measurement of LIC is recommended with chelation therapy as indicated.
Authors:
Khaled M Musallam; Maria D Cappellini; John C Wood; Ali T Taher
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Blood reviews     Volume:  26 Suppl 1     ISSN:  1532-1681     ISO Abbreviation:  Blood Rev.     Publication Date:  2012 Apr 
Date Detail:
Created Date:  2012-05-28     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8708558     Medline TA:  Blood Rev     Country:  England    
Other Details:
Languages:  eng     Pagination:  S16-9     Citation Subset:  IM    
Copyright Information:
Copyright © 2012 Elsevier Ltd. All rights reserved.
Affiliation:
IRCCS Ca' Granda Foundation Maggiore Policlinico Hospital, University of Milan, Milan, Italy.
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