Terpos Evangelos, Voskaridou Ersi
Department of Hematology & Medical Research, 251 General Air Force Hospital, Athens, Greece.
Pediatr Endocrinol Rev. 2008 Oct;6 Suppl 1:94-106.
Bone is an active tissue constantly being remodeled and changing metabolically through the balanced activity of osteoclasts, osteoblasts and osteocytes. Recent data have revealed the role of immune system to regulate bone cells and participate in the pathogenesis of several bone disorders. The RANK/RANKL/OPG system and the Wnt signaling are crucial pathways for the differentiation and function of osteoclasts and osteoblasts. Osteopenia and osteoporosis represent painful complications of thalassemia. Several factors that are responsible for bone loss in thalassemia act through the activation of osteoclasts and/or the inhibition of osteoblasts. Alterations in the RANKL/OPG ratio and increased serum levels of Wnt antagonists are present in thalassemic patients with osteoporosis. This review summarizes all available data for the interactions between bone and immune cells and their possible implications for the pathogenesis of bone loss in thalassemia. Through the understanding of the molecular pathways that are responsible for bone destruction in these patients we will be able to produce novel agents targeting these pathways and thus improve the management of osteoporosis in thalassemia.
骨骼是一种活跃的组织，通过破骨细胞、成骨细胞和骨细胞的平衡活动不断进行重塑并在代谢上发生变化。最近的数据揭示了免疫系统在调节骨细胞和参与几种骨疾病发病机制中的作用。RANK/RANKL/OPG系统和Wnt信号通路是破骨细胞和成骨细胞分化及功能的关键途径。骨质减少和骨质疏松是地中海贫血的痛苦并发症。地中海贫血中导致骨质流失的几个因素通过激活破骨细胞和/或抑制成骨细胞起作用。骨质疏松的地中海贫血患者存在RANKL/OPG比值改变和血清Wnt拮抗剂水平升高的情况。本综述总结了关于骨与免疫细胞相互作用的所有现有数据及其对地中海贫血骨质流失发病机制的可能影响。通过了解这些患者骨质破坏所涉及的分子途径，我们将能够研发针对这些途径的新型药物，从而改善地中海贫血骨质疏松的治疗。
