Abstract
Since the introduction of an improved blood transfusion and iron chelation therapy in patients with beta-thalassemia major (TM) and intermedia (TI), changes in bone structure and bone deformities are significantly diminished. However, older patients with thalassemia have a higher prevalence for fractures caused by osteoporosis. Additionally, most of these patients suffer from chronic bone pain.
In 13 patients (10 TM und 3 TI, age 8 – 32 years), we have measured the bone mineral density (BMD) by dual-energy x-ray absorption (DXA). All patients, except one, regularly received blood transfusions and iron chelation treatment (deferoxamine, deferiprone, or deferasirox). In the young patient group (n = 5, age: 8 -20 y), a mean Z-score of −1.53 was found (Z-score was calculated from age related BMD). In patients > 20 years (n = 8, age: 21 – 32 years), a mean T-score of −1.66 was observed (T-score was calculated from BMD related to 30 years). Except two patients, all patients had a decreased BMD in comparison to the normal population. One female patient with normal BMD, already received osteoporosis treatment since 5 years due to initially low BMD. Moreover, all patients had a reduced vertebra height in comparison to the normal population.
First results from monitoring the bone density by DXA and the trabecular bone structure by micro-CT, will be reported in all patients after 12 to 18 months under oral supplementation treatment with calcium and vitamin D.
Disclosure: No relevant conflicts of interest to declare.
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