Gupta Ritama, Musallam Khaled M, Taher Ali T, Rivella Stefano
Department of Pediatrics, Division of Hematology, Children's Hospital of Philadelphia (CHOP), 3401 Civic Center Boulevard, Philadelphia, PA 19104, USA.
International Network of Hematology, London WC1V 6AX, UK.
Hematol Oncol Clin North Am. 2018 Apr;32(2):213-221. doi: 10.1016/j.hoc.2017.11.009. Epub 2017 Dec 29.
Stress erythropoiesis (SE) is characterized by an imbalance in erythroid proliferation and differentiation under increased demands of erythrocyte generation and tissue oxygenation. β-thalassemia represents a chronic state of SE, called ineffective erythropoiesis (IE), exhibiting an expansion of erythroid-progenitor pool and deposition of alpha chains on erythrocyte membranes, causing cell death and anemia. Concurrently, there is a decrease in hepcidin expression and a subsequent state of iron overload. There are substantial investigative efforts to target increased iron absorption under IE. There are also avenues for targeting cell contact and signaling within erythroblastic islands under SE, for therapeutic benefits.
应激性红细胞生成(SE)的特征是在红细胞生成和组织氧合需求增加的情况下,红系增殖与分化失衡。β地中海贫血代表一种慢性SE状态,称为无效红细胞生成(IE),表现为红系祖细胞池扩大以及α链在红细胞膜上沉积,导致细胞死亡和贫血。同时,铁调素表达下降,随后出现铁过载状态。目前有大量研究致力于针对IE状态下增加的铁吸收。在SE状态下,也有通过靶向红细胞岛内的细胞接触和信号传导来获得治疗益处的途径。