Gonadal Hormones in Adolescent Females with β-Thalassemia in Relation to Iron Load.

BACKGROUND: Beta-thalassemia is 'a hereditary blood disorder characterized by reduced or absent beta globin chain synthesis, resulting in reduced hemoglobin in red blood cells, decreased RBCs production and anemia'. Patients with thalassemia major require repeated blood transfusions which 'lead to accumulation of iron in different tissues, including tissues of endocrine glands'. This study aims to evaluate serum gonadal hormones levels in adolescent females with β-thalassemia in relation to iron overload.

SUBJECTS AND METHODS: This study was conducted on 80 adolescent females with β-thalassemia having serum ferritin over 1000 ng/ml with range of their ages between 11 -15 years and mean age of 12. 42 ± 1.12 years (Group I) and 80 females with β-thalassemia of matched age having serum ferritin less than 500 ng/ml (Group II). For all patients the following were done: Complete blood count, hemoglobin electrophoresis, serum iron status including 'serum ferritin, serum iron and total iron binding capacity and' gonadal hormones including LH, FSH, and serum Estrogen.

RESULTS: 'There were significantly higher serum ferritin and serum iron and significantly lower TIBC', Follicular Stimulating Hormone, Luteinizing Hormone and Estrogen levels in Group I compared with Group II (Mean serum ferritin was 1839.5 ± 258.2 ng/ml in group I versus 336.2 ± 33.5 ng/ml in group II with p value of 0.001, mean serum iron was 201.3 ± 38.43 ug/dl in group I versus 124.47 ± 12.23 ug/dl in group II with p value of 0.001, mean serum total iron binding capacity was 252.56 ± 23.21 ug/dl in group I versus 353.6 ± 31.79 ug/dl in group II with p value of 0.001, mean FSH level was 1.17 ± 0.67 mIU/ml in group I versus 2.55 ± 1.92 mIU/ml group II with p value of 0.029, mean LH level was 0.98 ± 0.25 mIU/ml in group I versus 1.91 ± 0. 42 mIU /ml in group II with p value of 0.016, mean Estrogen level was 22.46 ± 6.36 pg/ml in group I versus 35 .63 ± 8.63 pg/ml in group II with p value of 0.010). There were significant negative correlations between gonadal hormones including serum Follicle-Stimulating Hormone, Luteinizing Hormone, Estrogen and serum ferritin (r = - 0. 835 and p value of 0.01 for FSH and serum ferritin, r = -0. 949 and p value of 0.01 for LH and serum ferritin and r= - 0. 900 and p value of p 0.01 for Estrogen and serum ferritin.

CONCLUSION: Female patients with β-thalassemia with iron overload may have gonadal hormones deficiency with significant negative correlation between gonadal hormones and serum ferritin.

RECOMMENDATIONS: Regular iron chelation to prevent iron overload with subsequent irreversible damage of the ovaries and also regular follow up for females with β-thalassemia with assessment of puberty as they are more vulnerable to develop hypogonadism and may require hormonal replacement therapy.