Choice of the Scheme for Blood Transfusion in Thalassemia Major:
- Intermediat schemes:
Mean Hb 9-10 gm/dL are acceptable in terms of daily living, they may have to be accepted in areas where the blood supply is inadequate (Cazzola et al., 1997).
- Hypertransfusion schemes :
Mean Hb 10 gm/dL or greater. Improve the quality of life without accelerating the lethal complications of iron overload, also suppress erythroid activity and prevent the unlimited bone marrow expansion that underlies the skeletal pathology of thalassemia major. Additional advantages of a regular transfusion program include prevention or delay in the development of congestive splenomegaly and fewer severe infectious illnesses (Lukens, 1993).
- Supertransfusion program:
Stabilizes Hb levels at 11 to 12 gm/dL. Hemoglobin value is never allowed to drop below 12 gm/dL and is raised regularly to 14 gm/dL by small transfusion every 2-3 weeks. Super-transfusion permits an excellent quality of life and suppression of the bone marrow so that abnormal cells are cleared from the peripheral blood (Model and Berdoukas,, 1984).
There is no doubt that the judicious use of chelation has been one of the most effective therapeutic advances in prolonging life of thalassemia patients (Deborash and Elizer, 2000). Patients who begin treatment at a young age can be protected from the lethal complications of iron overload for at least two decades. Those persons with established heart and other organ dysfunction may experience an arrest of disease progression or improvement of function with intense chelation (Cohen, 2006).
a. Subcutaneous Iron Chelators:
Desferoxamine B atrihydroxamic acid produced by streptomyces pilosus, with relative specificity for ferric iron. Desferoxamine is poorly absorbed from the gastrointestinal tract and has an extremely short half–life and thus it must be administered parenterally, usually a continuous subcutaneous infusion in a dose of 25-50 mg/kg given over 8-12 hours 5-7 days per week (Kwiatkowski, 2008).