MONITORING AND ESTIMATION OF THE EFFECTS OF BLOOD-TRANSFUSION AND IRON CHELATION IN THALASSEMIA MAJOR

  • 1 January 1982
    • journal article
    • research article
    • Vol. 24  (5) , 281-287
Abstract
A total of 21 patients with thalassemia major between 2 and 20 yr old were on a hypertransfusion regimen for 1-14 yr periods. The use of the transfusion quotient (TQ) allowed a precise survey of the blood requirement. In all splenectomized patients the TQ was between 1 and 2. TQ increased and was always > 2 when patients became hypersplenic. Of the 21 regularly transfused patients, 12 had Fe chelation therapy by continuous s.c. desferrioxamine (DF) injection (1 g over 8-12 h at home, nightly). The Fe balance was negative for 7 patients, equilibrated for 1 and positive for 4. All patients receiving more than weekly injections of DF had a negative balance; the variation of the serum ferritin level correlated with the Fe balance (r = 0.77; P < 0.01). The beneficial effects of the treatment were improvement in social and professional activity, in growth and puberty development and attenuation of the skull and face dysmorphia. Monitoring ferritin is a simple way of estimating the effects of chelation on Fe overload.

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