Receptor Activator of Nuclear Factor κ-Β Ligand/Osteoprotegerin Axis in Adults with Hb S/β-Thalassemia and β-Thalassemia Trait

• Published in: Hemoglobin 2020-09, Vol.44 (5), p.334-337
• Main Authors: Tombak, Anil, Boztepe, Burcu, Akbayir, Serin, Dogru, Gurbet, Sungur, Mehmet Ali
• Format: Article
• Language: English
• Subjects: Bone mineral density (BMD); osteoporosis; osteoprotegerin (OPG); receptor activator of nuclear factor κ-Β ligand (RANKL); sickle cell disease; thalassemia
• ISSN: 0363-0269; 1532-432X
• DOI: 10.1080/03630269.2020.1811116

There is not enough data about osteoporosis and the role of receptor activator of nuclear factor κ-Β ligand (RANKL)/serum osteoprotegerin (OPG) system in patients with double heterozygosity for sickle cell disease and β-thalassemia [Hb S (HBB: c.20A>T)/β-thal] and β-thal trait. Aim of the study was to investigate bone mineral densities (BMD) and the role of RANKL/OPG system in these cases. We studied 58 adults with Hb S/β-thal, 52 adults with β-thal trait, 34 healthy subjects as a control group. The BMD was determined by dual-energy X-ray absorptiometry (DEXA). Biochemical markers of bone metabolism (serum calcium, phosphorus, alkaline phosphatase, osteocalcin) parameters that affect bone metabolism (serum parathyroid hormone, thyroid-stimulating hormone, 25-hydroxyvitamin D, OPG, soluble RANKL [sRANKL]) were studied. Femoral neck Z-scores of 93.2% for β-thal trait, 83.0% for Hb S/β-thal patients were within the expected range. Lumbar spine Z-scores of 89.1% for β-thal, 90.2% for Hb S/β-thal patients were above −2.0 SD. Z-scores of the control group were within the expected range. Median serum sRANKL level was 2.80, 4.52, 5.79 pmol/L in Hb S/β-thal, β-thal trait, control groups, respectively (p = 0.010). Median serum OPG level was 1.07, 0.86, 0.86 pmol/L in Hb S/β-thal, β-thal trait, control groups, respectively (p