Y. Aydinok et al., Sequential use of deferiprone and desferrioxamine in primary school children with thalassaemia major in Turkey, ACT HAEMAT, 102(1), 1999, pp. 17-21
The effectiveness of the sequential use of deferiprone and desferrioxamine
(DFO) in children with thalassaemia major was examined. Seven thalassaemic
children in whom urinary iron induced by deferiprone was sufficient to main
tain a negative iron balance were enrolled in the long-term trial. Deferipr
one at a dose of 75 mg/kd/day in 3 divided doses was given for 4 school day
s a week. The group was given DFO at a dose of 40-50 mg/kg/day s.c. over 8-
12 h with a battery-operated pump for 2 days at the weekend. In addition to
the safety variables, they were monitored for serum ferritin levels at 2-m
onth intervals and hepatic iron concentrations in liver tissues were determ
ined at the beginning and the 6th month of therapy. The severity of hepatic
damage was graded according to the Knodell hepatic activity index and the
fibrosis was quantified. None of the patients suffered adverse effects of t
he therapy but a transient increase in serum ALT levels was noted. A nonsig
nificant decline in serum ferritin was observed (p = 0.08), a significant r
eduction in hepatic iron concentration was also determined (p = 0.03). The
hepatic activity index in liver tissues of the patients at the 6th month of
the sequential therapy significantly decreased (p = 0.03) whereas fibrosis
scores did not differ significantly (p = 0.25).