title: Association between bone mineral density and erythropoiesis in Thai children and adolescents with thalassemia syndromes.
abstract: Increased marrow erythropoiesis in patients with thalassemia syndromes results in the expansion of bone marrow cavities and consequently decreases bone tissues, leading to osteoporosis. Whether the soluble transferrin receptor (sTfR), a marker of erythropoietic activity, correlates with the bone mineral density (BMD) in thalassemic patients has not previously been addressed. Forty-six children and adolescents with thalassemia syndromes, who were either not transfused or suboptimally transfused, were studied. BMD was determined by dual-energy X-ray absorptiometry. Blood samples were obtained in order to determine sTfR and hemoglobin. The patients were categorized into four groups: 1, beta-thalassemia/hemoglobin E (beta-thal/E) with transfusion-dependency (TD) (n = 18); 2, beta-thal/E with transfusion-independency (TI) (n = 15); 3, beta-thalassemia major (beta-major) (n = 6); 4, hemoglobin H (HbH) (n = 7). All patients had normal serum free thyroxine (FT4) and thyroid-stimulating hormone (TSH), and intact parathyroid hormone (PTH), serum calcium (Ca), phosphate (P), and 25-OH-vitamin D levels. The BMD of patients in the beta-major and beta-thal/E with TD groups were not significantly different. In comparison with the beta-major and beta-thal/E with TD groups, the beta-thal/E with TI and HbH groups had significantly higher BMD of the total body (TB), femoral neck (FN), and lumbar spine (LS), as well as higher levels of hemoglobin. In contrast, the sTfR levels of the beta-major, beta-thal/E with TI, and HbH groups were significantly lower than those of the beta-thal/E with TD group. The BMD of TB, FN, and LS was negatively correlated with the sTfR level, but positively correlated with the hemoglobin level. In conclusion, increased marrow erythropoiesis is one of the major determinants of reduced bone mass in thalassemic patients with either no transfusion or suboptimal transfusion.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Rama 6 Road, Bangkok, 10400, Thailand. rapmw@mahidol.ac.th
authors.forename: Pat
authors.initials: P
authors.lastname: Mahachoklertwattana
authors.forename: Pensri
authors.initials: P
authors.lastname: Pootrakul
authors.forename: Ampaiwan
authors.initials: A
authors.lastname: Chuansumrit
authors.forename: Lulin
authors.initials: L
authors.lastname: Choubtum
authors.forename: Arporn
authors.initials: A
authors.lastname: Sriphrapradang
authors.forename: Rojana
authors.initials: R
authors.lastname: Sirisriro
authors.forename: Rajata
authors.initials: R
authors.lastname: Rajatanavin
mesh_terms: D000293:Adolescent; D001774:Blood Chemical Analysis; D001803:Blood Transfusion; D015519:Bone Density; D001854:Bone Marrow Cells / Q000378:metabolism; D002648:Child; D004920:Erythropoiesis; D005260:Female; D006801:Humans; D008297:Male; D015233:Models, Statistical; D011627:Puberty; D011990:Receptors, Transferrin / Q000378:metabolism; D013785:Thailand; D013789:Thalassemia / Q000175:diagnosis*; D013997:Time Factors; D014168:Transferrin / Q000096:biosynthesis
chemical_list: D011990:Receptors, Transferrin; D014168:Transferrin