In Cooley's anemia, maintenance of a hemoglobin level≥9.5 g/dl prevents anoxia and suppresses endogenous erythropolesis. Eight patients have been maintained on this transfusion regimer, since initial diagnosis, for 3 to 13 years. The patients have maintained excellent health and have been able to live normal lives, including school sports. Hepato-splenomegaly has been contained to a modest level; hyperspienism has not developed. Cardiac size and function have been normal; however, echo-cardiography revealed increased left ventricular wall thickness in children older than 8 years, suggesting increased Fe depostion. All children have received test doses of I.M. Desferrioxamine-B 20 mg/Kg 15-16 times each year. The results indicate that in the youngest children, 20% of the transfused Fe, and in the oldest children, 50%, could be eliminated by dally I.M. Injections. Recently, the same dose of Desferrioxamine-B has been administered by intravenous or subcutaneous infusion overnight. By these routes, the excretion of Fe has increased fourfold in the youngest, and twice in 1he oldest children.
Thus, children with Cooley's anemia may be kept in excellent health by this transfusion regimen, and the iror overload prevented by daily subcutaneous chelation therapy at home. This should result in increased longevity.
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Weiner, M., Karpatkin, M., Hart, D. et al. COOLEY'S ANEMIA: TRANSFUSIONS REGIMEN AND IPON BALANCE. Pediatr Res 11, 483 (1977). https://doi.org/10.1203/00006450-197704000-00679