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We studied bone mass and metabolism in 30 adult women (age 28.5 +/- 1.3) with thalassemia major (TM) and evaluated whether prolonged hormone replacement therapy (HRT) was able to optimize bone accrual. TM patients had reduced bone mass, increased bone turnover and lower serum gonadotropin and estradiol levels compared with 10 normal women of similar age. A significant correlation was found between bone mass and sex hormone levels. Six TM patients with normal ovarian function had normal bone turnover markers and modestly low bone mass (lumbar spine -1.29 +/- 0.31; femoral neck -0.60+/-0.21; Z-score). The other 24 TM women were hypogonadic and had significantly lower bone mass for age (lumbar spine -2.35 +/- 0.2, femoral neck -1.83 +/- 0.2) and increased bone turnover relative to eugonadal women. Of the hypogonadal patients, 13 had taken HRT since age 15 +/- 1 years, but their bone mass and turnover markers were not different than untreated hypogonadal patients. In conclusion, while hypogonadism negatively affects bone mass acquisition in adult TM women, HRT at the standard replacement doses is not sufficient to secure optimal bone accrual.

Remarkable advances in blood transfusion and iron-chelation therapy have improved and prolonged the survival of patients with thalassemic syndromes. Because of this increase in mean survival, new therapeutic strategies have been designed in order to limit the most important complications involving heart, liver and endocrine tissues. Endocrine disorders are rarely reported as cause of death in thalassemics but they can worsen the quality of life of these patients. Most authors have reported high incidence of endocrine abnormalities in thalassemics and the iron overload is frequently indicated as the most important pathogenetic factor [1]. De Sanctis et al. [2], in a multicenter study, reported that endocrine abnormalities involve more than 50% of thalassemic patients. Particularly the incidence of thyroid disorders has shown a large variability, according to the findings reported in the various studies [3–5]. The aim of this study was to evaluate the thyroid function in 58 thalassemic patients in order to evaluate possible correlations with age, serum ferritin values, and liver impairment.