Bone disease
Although RBC transfusions suppress IE, making skeletal abnormalities
less common today than in the past, bone health in thalassemia
patients must be monitored to identify age-related low bone mass.
Nearly 90% of TM patients, including 30% of those younger than
12 years, have low bone mass Z-score (_ 2.0).36 For this reason,
beginning in childhood, yearly studies that include bone mineral
density as well as studies of calcium, vitamin D3 metabolism, and
thyroid and parathyroid function should be performed
Low bone mass is associated with a high prevalence of
fractures in TM (17%) and TI (12%) patients, and the frequency
increases with age, hypogonadism, and increased bone turnover.36
Some short-term success has been seen with the administration of
pamidronate in patients with Z-/T-score _ 2.5. Important preventive
measures include age-appropriate calcium and vitamin
D supplementation and timely use of hormonal supplementation.1
It seems that early administration of iron chelation is effective
in preventing endocrine complications. According to the Thalassemia
Clinical Research Network, 96% of chelated thalassemia
patients with a median age of 20 years were free of hypoparathyroidism,
91% had no thyroid disease, and 90% were free of diabetes.
Overall, 62% were free of any endocrinopathy.37 However, this is
not always the case because some patients may develop endocrine
complications despite chelation.