Chronic erythrocyte transfusion therapy is the treatment of choice for certain hereditary anemias such as beta-thalassemia major and Diamond- Blackfan Anemia that is unresponsive to steroids. In addition, chronic transfusion therapy is recommended for patients with sickle cell disease who have experienced a cerebral vascular accident (CVA) in order to reduce the risk of recurrent CVA's, which is as high as 80% in untransfused patients. Transfusion therapy is also beneficial for sickle cell patients who suffer from severe, frequent vaso-occlusive crises or acute chest syndromes. This form of therapy has had a tremendous impact on the quality of life of these patients; however, this therapy is associated with many complications including iron overload, transmission of infecious diseases and alloimmunization. Transfusional iron overload is one of the major causes of morbidity & mortality in chronically transfused patients. As the total iron burden rises, iron depostition in the heart, liver, endocrine organs & the skin inevitably leads to organ dysfunction. Cardiac iron overload is the most serious consequence of transfusion therapy & cardiac complications are frequently the cause of death in the third or fourth decades for life of heavily transfused patients.

Project Start
2001-12-01
Project End
2002-11-30
Budget Start
Budget End
Support Year
41
Fiscal Year
2002
Total Cost
Indirect Cost
Name
Johns Hopkins University
Department
Type
DUNS #
045911138
City
Baltimore
State
MD
Country
United States
Zip Code
21218
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