Document Detail


The natural history of thalassemia intermedia.
MedLine Citation:
PMID:  20712795     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The severity of thalassemia intermedia depends on the degree of imbalance between alpha and non-alpha chains as well as other genetic and environmental factors that modify the natural history of the disease. By definition, the patients spontaneously maintain hemoglobin at or above 7 g/dL, sometimes at the price of intense hyperplasia of the bone marrow that is in turn responsible for bone deformities, osteoporosis, and extramedullary erythropoietic masses that often characterize thalassemia intermedia. Transfusion may become necessary with advancing age, during infection and pregnancy, and when hypersplenism develops. Splenectomy is often needed. Iron overload in nontransfused patients is due to increased gastrointestinal absorption and involves mainly the liver. Complications affecting the lives of patients with thalassemia intermedia include pulmonary hypertension, leg ulcers, pseudoxanthoma elasticum, gallstones, hepatocellular carcinoma, and thromboembolic events.
Authors:
Caterina Borgna-Pignatti; Maria Marsella; Nicolò Zanforlin
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Annals of the New York Academy of Sciences     Volume:  1202     ISSN:  1749-6632     ISO Abbreviation:  Ann. N. Y. Acad. Sci.     Publication Date:  2010 Aug 
Date Detail:
Created Date:  2010-08-17     Completed Date:  2010-09-17     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7506858     Medline TA:  Ann N Y Acad Sci     Country:  United States    
Other Details:
Languages:  eng     Pagination:  214-20     Citation Subset:  IM    
Affiliation:
Department of Clinical and Experimental Medicine, Università di Ferrara, Ferrara, Italy. bre@unife.it
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MeSH Terms
Descriptor/Qualifier:
Cardiovascular Diseases / etiology,  physiopathology
Cholelithiasis / etiology
Endocrine System Diseases / etiology,  physiopathology
Female
Hemoglobins / chemistry,  metabolism
Humans
Hypertension, Pulmonary / etiology,  physiopathology
Iron Overload / etiology,  physiopathology
Leg Ulcer / etiology
Pregnancy
Pseudoxanthoma Elasticum / etiology
Risk Factors
Thalassemia / complications,  pathology,  physiopathology*,  therapy
Thrombosis / etiology
Chemical
Reg. No./Substance:
0/Hemoglobins

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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