The aim of this study was to assess the level of
hepcidin in hereditary chronic hemolytic
anemias and to correlate the serum
hepcidin levels to the need for
blood transfusions (frequency of
blood transfusions and the serum
ferritin level). Seventy pediatric patients with hereditary chronic hemolytic
anemias, attending to hematology clinics of Cairo University and Misr University for Science and Technology (MUST) hospitals were the subjects of this study [53 patients with β-
thalassemia major (β-TM), 10 patients with β-
thalassemia intermedia (β-TI), four patients with
congenital spherocytosis and three patients with
sickle cell disease) (38 males and 32 females)]; their ages ranged from 1-14 years. Seventy normal children, age- and sex-matched, served as the control group. The results of this study revealed decreased
hepcidin levels in patients (all types of congenital chronic hemolytic
anemias) [mean ± SD (standard deviation) = 22.9 ± 6.0] compared to controls (mean ± SD = 132.4 ± 16.7) with highly significant statistical difference in between.
Hepcidin levels were higher in β-TM patients (mean ± SD = 23.7 ± 6.2) than in β-TI patients (mean ± SD = 21.8 ± 4.0), the
hepcidin to
ferritin ratio was significantly less than one. In β-TM patients, the mean ± SD was 0.03 ± 0.004, and in β-TI patients the mean ± SD = 0.025 ± 0.002, with highly significant statistical difference with
hepcidin-to-
ferritin ratios in controls being mean ± SD = 2.3 ± 0.7.
Hepcidin and
hepcidin/
ferritin ratios can be used as good markers of
hemolytic anemia and
iron overload as they have very high sensitivity (99.0 and 99.0%, respectively) and very high specificity (98.0 and 97.0%, respectively). Our findings highlight the potential usefulness of
hepcidin measurement as a diagnostic tool. The use of
hepcidin as an adjuvant
therapy with
iron chelators is important as it has a vital role in combating hemosidrosis.