Hemoglobinopathy Research Program, Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada.
Ann N Y Acad Sci. 2010 Aug;1202:155-7. doi: 10.1111/j.1749-6632.2010.05579.x.
Globally, hemoglobin (Hb) E beta thalassemia accounts for approximately half the severe forms of beta thalassemia. Because of its wide clinical diversity and the ability of patients with this condition to adapt unusually well to low hemoglobin levels, the management of Hb E beta thalassemia, particularly the decision to instigate regular blood transfusion, is particularly difficult. Here, we present a summary of our work in patients with this condition, which attempts to define clinical, adaptive, and genetic factors of possible value in determining the early management of this condition.
在全球范围内，血红蛋白 E 地中海贫血症约占β地中海贫血症严重形式的一半。由于其广泛的临床多样性以及患者适应低血红蛋白水平的异常能力，Hb E 地中海贫血症的管理，特别是决定是否进行定期输血的决策，尤其困难。在这里，我们总结了我们在这些患者中的工作，试图确定可能有助于确定这种疾病早期管理的临床、适应和遗传因素。
