BONE-DENSITY, MINERAL-CONTENT, AND CORTICAL INDEX IN PATIENTS WITH THALASSEMIA MAJOR AND THE CORRELATION TO THEIR BONE-FRACTURES, BLOOD-TRANSFUSIONS, AND TREATMENT WITH DESFERRIOXAMINE

In an attempt to explain the increased frequency of pathological fractures found in thalassemia major patients, we examined noninvasively several properties of bones in 17 patients. We found a significant decrease in the bone mineral content (BMC) and the bone density (BD) of both trabecular (34% and 4% diminution, respectively) and cortical bone (24% and 3% diminution, respectively), as compared with normal controls. We also found that metacarpal cortical indices (CI) were significantly lower in thalassemic patients as compared with normal controls (36% diminution). As expected, upper limb fractures were more frequent in those patients with greater bone diminution. Furthermore, the degree of bone diminution was greater in patients receiving fewer blood transfusions and shorter courses of desferrioxamine. Bone densitometry should probably be included in the comprehensive evaluation of thalassemia patients in order to help guide overall treatment.