A) T. Major (Cooley’s anemia) A group of inherited disorders caused by underproduction of alpha or beta-globin chains of Hb molecule leading to deficient hemoglobin accumulation with hypochromic, microcytic red cells.
Treatment
Needs Hematologist supervision. Packed cell transfusions to keep Hb between 10-14 gms/dl. Usually required at intervals of 3-4 weeks life long. Chelating agent deferoxamine mesylate subcutaneously by infusion pump for lifetime to avoid hemochromotosis. Oral Deferoxamine mesylate subcutaneously by infusion pump for lifetime to avoid hemochromotosis. Oral chelating agent Defreprone (75 mg/kg) may also be given as an alternative. Splenectomy may be required after the age of 7, if the need for transfusion increases.
A) T. Minor
Only folic acid 5 mg/week life long. No iron is given unless there is proven iron deficiency, or during pregnancy when the anemia does not respond to folic acid.
