Hemoglobin E beta (ß)-thalassemia (HbE thalassemia) is a very common form of β-thalassemia that exhibits a heterogeneous clinical presentation and variable clinical course. The reasons for this extraordinary clinical heterogeneity are not completely understood. A number of factors, both genetic and environmental, appear to modify the severity of HbE thalassemia. There is also an emerging understanding that the HbE thalassemia phenotype may be unstable, which may reflect changes in adaptation to anemia and, possibly, attenuation of the erythropoietin response over time. These factors make it difficult to develop broad treatment guidelines. It is now generally appreciated that steady-state hemoglobin concentration may be of limited value to determine which patients need regular transfusions. Therefore, periodic reassessment of the need for transfusion therapy is recommended, and intermittent transfusion therapy may now be explored as an approach in this disorder.

中文翻译：

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血红蛋白Eβ地中海贫血的治疗策略。

血红蛋白Eβ（地中海贫血）地中海贫血（HbE地中海贫血）是β地中海贫血的一种非常常见的形式，表现出异质的临床表现和可变的临床过程。这种异常的临床异质性的原因尚不完全清楚。遗传和环境因素均可能改变HbE地中海贫血的严重程度。还出现了一种新的认识，即HbE地中海贫血表型可能是不稳定的，这可能反映了对贫血适应性的变化，并可能反映了促红细胞生成素反应随时间的减弱。这些因素使制定广泛的治疗指南变得困难。现在通常认识到稳态血红蛋白浓度对于确定哪些患者需要定期输血可能具有有限的价值。所以，