Abstract：
Recently, there has been an increasing number of studies on the crosstalk between the bone and the bone marrow and how it pertains to anemia. Here, we discuss four heritable clinical syndromes contrasting those in which anemia affects bone growth and development, with those in which abnormal bone development results in anemia, highlighting the multifaceted interactions between skeletal development and hematopoiesis.
Anemia results from both inherited and acquired disorders caused by either impaired production or premature destruction of red blood cells or blood loss. The downstream effects on bone development and growth in patients with anemia often constitute an important part of their clinical condition. We will discuss the interdependence of abnormal bone development and growth and hematopoietic abnormalities, with a focus on the erythroid lineage. To illustrate those points, we selected four heritable anemias that arise from either defective hematopoiesis impacting the skeletal system (the hemoglobinopathies β-thalassemia and sickle cell disease) versus defective osteogenesis resulting in impaired hematopoiesis (osteopetrosis). Finally, we will discuss recent findings in Diamond Blackfan anemia, an intrinsic disorder of both the erythron and the bone. By focusing on four representative hereditary hematopoietic disorders, this complex relationship between bone and blood should lead to new areas of research in the field.
Anemia results from both inherited and acquired disorders caused by either impaired production or premature destruction of red blood cells or blood loss. The downstream effects on bone development and growth in patients with anemia often constitute an important part of their clinical condition. We will discuss the interdependence of abnormal bone development and growth and hematopoietic abnormalities, with a focus on the erythroid lineage. To illustrate those points, we selected four heritable anemias that arise from either defective hematopoiesis impacting the skeletal system (the hemoglobinopathies β-thalassemia and sickle cell disease) versus defective osteogenesis resulting in impaired hematopoiesis (osteopetrosis). Finally, we will discuss recent findings in Diamond Blackfan anemia, an intrinsic disorder of both the erythron and the bone. By focusing on four representative hereditary hematopoietic disorders, this complex relationship between bone and blood should lead to new areas of research in the field.
摘要：
目标：最近，关于骨骼和骨髓之间的串扰以及它与贫血的关系的研究越来越多。这里,我们讨论了四种遗传性临床综合征，对比贫血影响骨骼生长和发育的那些，那些骨骼发育异常导致贫血的人，突出骨骼发育和造血之间多方面的相互作用。
结果：贫血是由红血球生产受损或过早破坏或失血引起的遗传性和获得性疾病引起的。对贫血患者骨发育和生长的下游影响通常构成其临床状况的重要部分。我们将讨论异常骨骼发育和生长与造血异常的相互依存关系，专注于红系谱系。为了说明这些观点，我们选择了4种遗传性贫血,它们是由影响骨骼系统的造血缺陷(血红蛋白病β-地中海贫血和镰状细胞病)和导致造血功能受损的成骨缺陷(骨结石)引起的.最后,我们将讨论DiamondBlackfan贫血的最新发现，erthron和骨的内在障碍。通过重点研究四种代表性的遗传性造血疾病，骨骼和血液之间的这种复杂关系将导致该领域的新研究领域。
结果：贫血是由红血球生产受损或过早破坏或失血引起的遗传性和获得性疾病引起的。对贫血患者骨发育和生长的下游影响通常构成其临床状况的重要部分。我们将讨论异常骨骼发育和生长与造血异常的相互依存关系，专注于红系谱系。为了说明这些观点，我们选择了4种遗传性贫血,它们是由影响骨骼系统的造血缺陷(血红蛋白病β-地中海贫血和镰状细胞病)和导致造血功能受损的成骨缺陷(骨结石)引起的.最后,我们将讨论DiamondBlackfan贫血的最新发现，erthron和骨的内在障碍。通过重点研究四种代表性的遗传性造血疾病，骨骼和血液之间的这种复杂关系将导致该领域的新研究领域。
