Abstract
IntroductionSickle cell anemia and thalassemia result in impaired bone health in both adults and youths. Children with other types of chronic hemolytic anemia may also display impaired bone health.Study DesignTo assess bone health in pediatric patients with chronic hemolytic anemia, a cross-sectional study was conducted involving 45 patients with different forms of hemolytic anemia (i.e., 17 homozygous sickle cell disease and 14 hereditary spherocytosis patients). Biochemical, radiographic and anamnestic parameters of bone health were assessed.ResultsVitamin D deficiency with 25 OH-vitamin D serum levels below 20 ng/ml was a common finding (80.5%) in this cohort. Bone pain was present in 31% of patients. Analysis of RANKL, osteoprotegerin (OPG) and osteocalcin levels indicated an alteration in bone modeling with significantly elevated RANKL/OPG ratios (control: 0.08+0.07; patients: 0.26+0.2, P = 0.0007). Osteocalcin levels were found to be lower in patients compared with healthy controls (68.5+39.0 ng/ml vs. 118.0+36.6 ng/ml, P = 0.0001). Multiple stepwise regression analysis revealed a significant (P<0.025) influence of LDH (partial r2 = 0.29), diagnosis of hemolytic anemia (partial r2 = 0.05) and age (partial r2 = 0.03) on osteocalcin levels. Patients with homozygous sickle cell anemia were more frequently and more severely affected by impaired bone health than patients with hereditary spherocytosis.ConclusionBone health is impaired in pediatric patients with hemolytic anemia. In addition to endocrine alterations, an imbalance in the RANKL/OPG system and low levels of osteocalcin may contribute to this impairment.
Highlights
Sickle cell anemia and thalassemia result in impaired bone health in both adults and youths
Analysis of receptor activator of nuclear factor kappa-B ligand (RANKL), osteoprotegerin (OPG) and osteocalcin levels indicated an alteration in bone modeling with significantly elevated RANKL/OPG ratios
Intrinsic hemolytic anemia can be subclassified as membranopathy, hemoglobinopathy or enzymopathy
Summary
Sickle cell anemia and thalassemia result in impaired bone health in both adults and youths. Children with other types of chronic hemolytic anemia may display impaired bone health. Chronic hemolytic anemia is a rare condition characterized by an abnormal breakdown of erythrocytes. Intrinsic hemolytic anemia can be subclassified as membranopathy (e.g., spherocytosis), hemoglobinopathy (e.g., sickle cell disease) or enzymopathy (e.g., pyruvate kinase deficiency). Extrinsic hemolytic anemias are caused by conditions such as autoimmunity or microangiopathy. Impaired bone health has been described in patients with sickle cell disease and thalassemia. These patients display altered parameters of bone metabolism and bone mineral density [2,3,4,5]
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