Bone mineral density in children with thalassaemia major: determining factors and effects of bone marrow transplantation

• Published in: Bone marrow transplantation (Basingstoke) Vol. 36; no. 4; pp. 331 - 336
• Main Authors: LEUNG, T. F, HUNG, E. C. W, LAM, C. W. K, LI, C. K, CHU, Y, CHIK, K. W, SHING, M. M. K, LEE, V, YUEN, P. M. P
• Format: Journal Article
• Language: English
• Published: Basingstoke Nature Publishing Group 01-08-2005
• Subjects: Adolescent; Adult; Alkaline phosphatase; Anemias. Hemoglobinopathies; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; beta-Thalassemia - epidemiology; beta-Thalassemia - physiopathology; beta-Thalassemia - therapy; Biological and medical sciences; Biomarkers - urine; Biomedical materials; Blood diseases; Bone Density; Bone marrow; Bone Marrow Transplantation; Bone marrow, stem cells transplantation. Graft versus host reaction; Bone mineral density; Bones; Care and treatment; Chemiluminescence; Child; China - epidemiology; Cross-Sectional Studies; Crosslinking; Density; Deoxypyridinoline; Diagnosis; Diseases of red blood cells; Dual energy X-ray absorptiometry; Enzyme immunoassay; Female; Hematologic and hematopoietic diseases; Hip; Humans; Immunoassay; Male; Medical sciences; Osteocalcin; Osteopenia; Osteoporosis; Patient outcomes; Predictive Value of Tests; Risk Factors; Spectrophotometry; Spine; Stem cell transplantation; Thalassemia; Transfusion; Transfusions. Complications. Transfusion reactions. Cell and gene therapy; Transplantation; Transplants & implants; X-Rays; China; Human; Hemoglobinopathy; biochemical marker; Hematology; Stem cell; Hematopoietic cell; Homograft; Biological marker; Hemopathy; Biochemistry; Genetic disease; thalassaemia major; Hemolytic anemia; β-Thalassemia; children; Bone marrow; Graft; Bone mineral density; Child
• ISSN: 0268-3369; 1476-5365
• DOI: 10.1038/sj.bmt.1705053

Osteoporosis and osteopenia affect up to half of patients with thalassaemia major (TM). We investigate the effects of acquired factors and BMT on bone mineral density (BMD) in these patients. In all, 53 patients on regular transfusion (BT group) and 33 patients at 5.7+/-1.9 years post transplant (BMT group) were recruited. BMD was measured by dual energy X-ray absorptiometry. Serum concentrations of osteocalcin, bone-specific alkaline phosphatase (ALP), beta-crossLap and urinary cross-linking deoxypyridinoline (DPD) were measured by chemiluminescence and enzyme immunoassay, respectively. Severe BMD deficit (Z-score <-2.5) at spine and hip were noted in 62 and 35% of BT group. Serum osteocalcin (beta=-0.463; P=0.006) was predictive of spine BMD, whereas age (beta=-0.843; P=0.007) and urine DPD (beta=-0.439; P=0.037) were associated with hip BMD in BT group. Among BMT patients, post transplant duration (beta=0.450; P=0.009) and serum bone-specific ALP (beta=-0.495; P=0.013) were associated with spine BMD. Severe BMD deficit was less common among BMT than BT patients (6 vs 35%; P=0.036). The mean (s.d.) osteocalcin levels in BMT and BT groups were 96.4 (72.7) microg and 68.9 (40.3) microg/l, respectively (P=0.037). In conclusion, severe BMD deficit is common in Chinese TM patients and BMT may reverse BMD deficit in these patients.