
HbF in HbE/β-thalassemia: A clinical and laboratory correlation
Just after birth, the level of HbF decreases gradually to <1%, and is replaced mainly by adult hemoglobin (HbA) (∼ 97%). However, higher HbF levels could be associated with HbE/β-thalassemia, a complex thalassemia intermedia with a diverse clinical severity ranging from mild-to-severe anemia.
指标解读:检验指标临床解读(18)血红蛋白异常性贫血-MedSci.cn
胎儿血红蛋白(HbF)酸洗脱试验 (fetal hemoglobi acid washing test) 正常值. 脐带血呼所有的红细胞均呈阳性,新生儿阳性率为55%~85%,1个月后的婴儿为67% 4~6个月后偶见成人小于1%。 影响因素. 1. 应严格掌握缓冲液的pH、酸洗脱的温度和时间,以保证测定结果的准确性。 2.
Why Are Hemoglobin F Levels Increased in HbE/β Thalassemia?
1999年11月1日 · These data suggest that the high HbF levels in HbE/β thalassemia, and other β thalassemia syndromes, result from increased erythropoietin levels leading to bone marrow expansion, and possibly increased F-cell production, combined with ineffective erythropoiesis giving a survival advantage to F cells.
Molecular understanding of unusual HbE-β + -thalassemia with …
Low HbF expression in HbE-β + -thalassemia may lead to misdiagnosis of HbE heterozygosity. We aimed to characterize the β - and α-globin genes and the modifying factors related to HbF expression in patients with an Hb phenotype similar to that of HbE heterozygotes.
The Hemoglobin E Thalassemias - PMC
Hemoglobin E (HbE) is a common structural β-hemoglobin variant. Although HbE alone does not cause significant clinical problems, its interactions with other thalassemias produce syndromes of varying severity. As discussed by Williams and Weatherall (2012), HbE occurs at an extremely high frequency in many countries in Asia.
Why are hemoglobin F levels increased in HbE/beta thalassemia?
1999年11月1日 · These data suggest that the high HbF levels in HbE/beta thalassemia, and other beta thalassemia syndromes, result from increased erythropoietin levels leading to bone marrow expansion, and possibly increased F-cell production, combined with ineffective erythropoiesis giving a survival advantage to F cells.
Genetic modifications of EGLN1 reactivate HbF production in β0 ...
2024年9月30日 · Reactivation of fetal hemoglobin (HbF, α 2 γ 2) potentially alleviates clinical presentation in β-thalassemia. Prolyl hydroxylase domain enzymes (PHDs) play roles in the canonical oxygen-sensing pathway and maintain the stability of cellular hypoxia-inducible factor α (HIF-α) in response to low oxygen levels or hypoxia.
However, higher HbF levels could be associated with HbE/β-thalassemia, a complex thalassemia intermedia with a diverse clinical severity ranging from mild-to-severe anemia. This study investigates the
检验指标解读 | 血红蛋白异常性贫血_全国体外诊断网(全国实验医 …
HbF轻度增高可见于:50%的轻型β-珠蛋白生成障碍性贫血、再生障碍性贫血(AA)、阵发性睡眠性血红蛋白尿(PNH)、真性红细胞增多症、铁粒幼细胞贫血、白血病等。 显著增高见于:重型珠蛋白生成障碍性贫血;α、F、A2F和γ-珠蛋白生成障碍性贫血及HbH病患者HbF也可增高;δ-珠蛋白生成障碍性贫血HbF不升高。 血红蛋白电泳. (hemoglobin electrophoresis) 正常血红蛋白电泳区带:HbA>95%,HbF<2%,HbA2为1%~3. 1%。 pH8. 6的TEB缓冲液适合于检出 HbA …
人的胎儿型血红蛋白(HbF)与成人型血红蛋白(HbA)在与氧分 …
蓝色线:HbF婴儿血红蛋白氧离曲线;绿色线:HbA成人血红蛋白氧离曲线;红色线:Mb肌红蛋白氧离曲线. 从氧离曲线可以看到,HbF对氧的亲和能力的确高于HbA (随氧分压上升更快达到饱和,达到50%饱和度时的氧分压分别是19和26.8)
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