leptin and beta-Thalassemia

The adipocytokines leptin and adiponectin represent a critical link between metabolism, immunity and chronic inflammation. A chronic vascular inflammatory state plays an important role in the pathophysiology of thalassaemia. We aimed to analyze the levels of these adipocytokines and determine any possible correlations with disease severity or vascular inflammation markers in beta-thalassaemia.. Serum leptin, adiponectin, high-sensitivity C-reactive protein, endothelins, vascular adhesion molecule-1, intracellular adhesion molecule-1 and L- and E-selectin were measured in 28 beta-thalassaemia patients and compared with levels in healthy controls.. Leptin was significantly lower in patients compared to controls (2.23 ± 1.8 vs. 10.24 ± 5.78 μg/l; p = 0.0018), whereas adiponectin was elevated (11.75 ± 5.67 vs. 6.83 ± 2.75 μg/l; p = 0.009). For both adipocytokines, no correlations were found with characteristics such as age, gender, type of chelation, body mass index z score or haemoglobin. Leptin, but not adiponectin, was negatively correlated with ferritin (p = 0.032, r = -0.61). No correlations were found between leptin and the inflammation markers. However, adiponectin was positively correlated with endothelin-1 (p = 0.022, r = 0.63).. Serum leptin is low in beta-thalassaemia, perhaps due to the toxic effect of iron overload on adipose tissue. Paradoxically, adiponectin levels are high and positively correlated with endothelin-1, raising questions about the pro- or anti-inflammatory role of this adipocytokine in beta-thalassaemia.

Topics: Adipokines; Adiponectin; Adolescent; Adult; Aging; beta-Thalassemia; Biomarkers; Blood Transfusion; Body Mass Index; C-Reactive Protein; Chelating Agents; Child; Endothelin-1; Endothelin-3; Female; Ferritins; Humans; Inflammation; Intercellular Adhesion Molecule-1; Leptin; Male; Middle Aged; Vascular Cell Adhesion Molecule-1

Patients with thalassemia major often present endocrine abnormalities due to dysfunction in their hypothalamic-pituitary axis. Leptin, an adipocyte derived hormone, primarily acts in hypothalamus and its deficiency in the ob/ob mouse results in persistent immaturity of its hypothalamic-pituitary function. The aim of the study was to evaluate leptin levels in thalassemic patients.. The study involved 33 adult patients (11 males), mean age (SD) 19.3 years (4.4) and a group of 12 prepubertal boys, mean age (SD): 7 years (1.7) with homozygous beta-thalassemia.. Mean (SD) leptin concentration was 3.2 (3.3) ng/mL in thalassemic males and 8.6 (3.3) ng/mL in thalassemic females; values significantly lower than matched normal subjects. In the group of prepubertal thalassemic children, leptin levels were also lower compared with matched healthy children, although the difference was not statistically significant.. In conclusion, low leptin levels were observed in thalassemic patients, which may be due to a toxic effect of iron to adipocytes.

Topics: Adolescent; Adult; beta-Thalassemia; Child; Female; Humans; Leptin; Male

The aim of the study was to investigate the association of leptin with hematological parameters in beta-thalassemia patients in Greece. We measured plasma levels of soluble transferrin receptor (sTfR) and leptin by enzyme-linked immunosorbent assay (ELISA) in 40 beta-thalassemia patients (21 transfusion dependent and 19 not transfused or sporadically transfused), in 20 beta-thalassemia carriers, and in 30 healthy individuals (HI). The percentage of reticulocytes (RET) was measured by the NE 9500 Sysmex automated method. Body mass index (BMI) was calculated by dividing body weight (kg) by square height (m). Endocrine measurements including sex hormones were also determined. sTfR concentrations were significantly higher in both transfusion-dependent (females 10.5+/-2.9, males 9.1+/-3.1) and non-transfusion-dependent patients (females 15.8+/-5.4, males 19.8+/-13.7) as compared to carriers (females 3.1+/-2.5, males 3.8+/-1.8) and to HI (females 1.5+/-1.2, males 2.5+/-2.1). Leptin levels were lower both in female and in male transfusion-dependent patients (0.5+/-0.3 and 1.2+/-1, respectively) and in non-transfused males (1.9+/-2) compared to carriers (females 7.9+/-2.7, males 13.1+/-9.1) and HI (females 14.6+/-6, males 7.5+/-3). There was a negative correlation between leptin and sTfR levels in transfused patients (R=-0.61, p<0.05). A stronger negative correlation (R=-0.7, p=0.006) was found in hypogonadic men and women with beta-thalassemia. These findings enhance previous results indicating that leptin may play some role in hematopoiesis and could associate the pathophysiology of thalassemic patients with the triggering effect of leptin in reproductive ability.

Topics: Adult; beta-Thalassemia; Case-Control Studies; Female; Greece; Heterozygote; Homozygote; Humans; Hypogonadism; Leptin; Male; Middle Aged; Receptors, Leptin; Receptors, Transferrin; Solubility; Statistics, Nonparametric

Topics: beta-Thalassemia; Female; Humans; Leptin; Male; Proteins