Changes of Bone Density in Pediatric Patients with β-thalassemia Major after Allogenic Hematopoietic Stem Cell Transplantation.
ABSTRACT Thalassemia major and its treatment by stem cell transplantation can have deleterious effects on bone integrity. This study assesses the adverse effects of transplantation on growing bones of pediatric thalassemic patients.
Bone mineral density (BMD) of 20 patients from three thalassemia classes whose mean (SD) age was 7.4 (3.8) years were tested with a Norland XR-46 device at baseline (before transplantation), 6 and 12 months after transplantation.
At 6 and 12 months after transplantation we observed no significant changes in mean BMD. There were no Z-scores less than -2 among patients. Class 3 thalassemia did not negatively impact BMD. Calcium (Ca), phosphorous (P) and ferritin levels were not significantly related to patients' BMD scores. Transfusion duration and chelation therapy showed positive significant relationships to BMD (g/cm2), but no significant relation with the BMD Z-score. The deleterious relation between corticosteroid use and changes in BMD was not significant. In contrast, patients who developed acute graft versus host disease (aGVHD) after transplantation showed significant adverse effects on BMD of their femur (P = 0.020) and spine (P = 0.027).
Stem cell transplantation in pediatric thalassemic patients who do not develop aGVHD does not appear to have any significant positive or negative effects on BMD.
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ABSTRACT: Low BMDs, short stature, hypogonadism, subclinical hypothyroidism, and IFG are found in 3.3, 10, 33, 16.6, 6.6, and 26.6 % of 30 pediatric β thalassemia major patients, respectively. Age is related with low Z-scores. Short stature and hypogonadism patients were older. These patients' monitoring in late childhood and early teenage for these complications is recommended. Beta-thalassemia major patients frequently have low BMD and increased fracture risk. We tried to determine the relation between BMD and biochemical, transfusion, and endocrinological parameters in pediatric patients. Thirty beta-thalassemia major patients entered the study. Male to female ratio was 14/16. Physicians collected demographic; anthropometric; menstrual; transfusion and treatments histories; and serum levels of calcium, phosphorus, alkalin phosphatase, FBS, Hb, Ferritin, T3, T4, TSH, LH, FSH, testosterone (for boys), and estradiol (for girls). BMD of the spine and femur was measured using a DXA Norland XR-46 device. Prevalence of "low bone density" (Z-score < -2) in the spine and femur was 3.3 and 10 %, respectively. Short stature (Z-score < -2.5) and hypogonadism was seen in 33 and 16.6 % of patients, respectively. Hypogonadism was found only in boys. Subclinical hypothyroidism and impaired fasting glucose were found in 6.6 and 26.6 % of our patients, respectively. Age and transfusion duration was positively related with spinal BMD (P values, <0.001 and <0.001, respectively), and both related negatively with spinal Z-score (P values, 0.014 and 0.043, respectively). Age and transfusion duration related positively with femoral BMD (P values 0.030 and 0.017, respectively), and both related negatively with femoral Z-score (P values, 0.015 and 0.041, respectively). Mean age of short stature and hypogonadism patients were significantly higher than those who were normal (P values, 0.007 and 0.044, respectively). The main factor related with lower Z-scores of the femur and spine was age. Short stature and hypogonadism patients were significantly older. So, we recommend early monitoring of thalassemia patients (in their late childhood and early teenage) for these complications.Archives of Osteoporosis 12/2014; 9(1):174. DOI:10.1007/s11657-014-0174-3