Thalassemia and bone changes

Abstract

In Beta-Thalassemia Major or Cooley's Anemia, the accelerated rate of destruction of erythroid cells both in the medulla and in the periphery, strongly stimulating the proliferation and maturation of erythroblasts through renal erythropoietin. Therefore, there is a great expansion of the bone marrow, more than in any other anemia, leading to the classic thalassemic bone deformities, such as: prominence of the jaws (thalassemic face or squirrel face), enlargement of the upper dental arch, with separation of the teeth and frontal bossing. Patients also present with other bone manifestations, such as: pain, growth retardation and bone age, scoliosis, spinal cord compressions, papathological fractures, osteopenia, and osteoporosis. However, osteopenia and osteoporosis seem to be caused by hormonal issues such as hypothyroidism, hypoparathyroidism, diabetes mellitus and hypogonadism, which cause an imbalance in bone remodeling.  Objective: To address how thalassemia major acts on bone changes. Method: This is a systematic review of the literature. It was prepared from a bibliographic survey, covering national scientific articles and books, in the last ten years. The databases used were SciELO, Google Scholar, Journal of Pediatrics and pathology books. Discussion: Thus, thalassemia can be correlated with bone loss in children, in addition to chronic anemia and hypoxia associated with pubertal and weight delay, making it lethal in this age group. Conclusion: this study confirms that there is severe bone loss in children with thalassemia major, which reinforces the importance of efficient diagnosis and treatment to improve the quality of life of these patients.

DOI:https://doi.org/10.56238/sevened2023.004-055