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).
Chelation
Therapy:
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:
-
Desferrioxamine:
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).
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