Endocrine Abstracts

Thalassaemia patients are transfusion dependent and at risk of iron overload with end organ damage. Iron deposition is reduced by chelation treatment. We present the data on endocrinopathy and bone density in the adult patients under our care with thalassaemia. In addition, we will discuss therapeutic approaches to these problems including the use of newer chelation agents and possible reversal of endocrine end organ damage. We will also review the correlation in our patients between the degree of endocrinopathy and their chelation status/ferritin and cardiac T* weighted MRI.

In our cohort of 19 predominantly South-East Asian patients, 15 have beta thalassaemia major (7 male, 8 female), 2 females have thalassaemia intermedia and 2 females have beta thalassaemia/HbE. The mean age of the males is 35 years (22–41) and females 32.9 years (19–50).

All of the patients with thalassaemia major and thalassaemia/HbE have hypogonadism. GH deficiency, hypothyroidism, hypoparathyroidism and adrenal insufficiency are present in varying degrees within this group. Eighty-five percent of patients are vitamin D deficient.

The majority of these young adults have failed to achieve peak bone mass. In the males, mean DEXA T score at the lumbar spine is −3.53 (−2 to −5.4) and femoral neck is −2.37 (−1.8 to −3). In the females, mean DEXA T score at the lumbar spine is −2.46 (0.4 to −4.2) and at the femoral neck is −1.67 (−0.1 to −3.2).

Advances in treatment mean that these patients are surviving into adulthood and will need long term endocrine care. There are few studies in adult compared to paediatric patients with thalasseamia and the data presented provides some insight into the management challenges.