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.

Agency
National Institute of Health (NIH)
Institute
National Center for Research Resources (NCRR)
Type
General Clinical Research Centers Program (M01)
Project #
3M01RR000052-38S1
Application #
6297533
Study Section
Project Start
1998-12-01
Project End
1999-11-30
Budget Start
1998-10-01
Budget End
1999-09-30
Support Year
38
Fiscal Year
1999
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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