Predictors of Osteopathy among Adult Patients with Thalassemia Major

Background: Beta thalassemia comprises a group of inherited (autosomal recessive) hematologic disorders characterized by decreased or absent synthesis of β-globin chains. Beta thalassemia patients exhibit an unbalance in bone mineral turnover with increased resorptive rates and suppression of osteoblast activity, resulting in diminished bone mineral density (BMD).

The Aim of Study: The aim of study was to determine the prevalence of low BMD in patients with β-thalassemia and to find a possible relationship between osteopathy among adult thalassemic patients and multiple variables as different endocrinal disorders, iron chelation therapy, calcium and vitamin D levels, ferritin levels and history of splenectomy.

Subjects and Methods: The current study had been conducted on 80 patients with β-thalassemia major. Thirty seven were males (46.2%) and 43 patients were females (53.8%). Their ages ranged from 15 to 33 years with a mean of 20.6 ± 4 years. All patients were subjected to full medical history, full clinical examination and investigations that included total calcium, 25 (OH) vitamin D, FBG, 2hPPBG, TSH, free T4 and ferritin levels. Assessment of BMD was done by DEXA scan at three sites (AP-spine, left femur and right forearm).

Results: Seventy-nine (98.7%) patients had abnormal BMD where 34 (42.5%) patients were osteoporotic, 6 (7.5%) were osteopenic and 39 (48.7%) had both osteopenia and osteoporosis. Bone mineral density positively correlated with vitamin D levels at left femur and AP spine, and Calcium levels at RT forearm, LT femur and AP spine. BMD negatively correlated with 2-hour postprandial blood glucose and ferritin levels at RT forearm, LT femur and AP spine. There was a statistically significant difference with p-value of 0.02 between number of sites affected and history of splenectomy, where (40.6%) of these who underwent splenectomy had affection in the three sites.

Conclusions: The prevalence of low BMD in patients with β-thalassemia is high. Many factors contribute to the development of such complication. BMD is a good index of bone status in patients with thalassemia. Regarding the high prevalence of osteopenia/osteoporosis in patients with thalassaemia major, all patients should be screened periodically for bone disease. Future studies including larger number of patients with different types of hemoglobinopathies are recommended.