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Hemoglobina Fetal (HbF): Estrutura, Função e Significado Clínico
Fetal hemoglobin (HbF, α2γ2) is the primary oxygen carrier during fetal life, replacing embryonic hemoglobins by 10–12 weeks gestation.
Key Properties
- Structure: Two α-globin and two γ-globin chains (vs. adult HbA: α2β2).
- High oxygen affinity: Left-shifted dissociation curve due to low 2,3-BPG binding—facilitates placental oxygen transfer.
- Alkaline resistance: Stable in the fetal alkaline environment.
- Production timeline: Liver/spleen → bone marrow; declines postnatally to <1% by 6–12 months.
Clinical Relevance
- Hereditary persistence of HbF (HPFH): Benign condition with elevated HbF in adulthood.
- Therapeutic induction: Hydroxyurea increases HbF in sickle cell disease and β-thalassemia, reducing sickling and globin imbalance.
- Diagnostic marker: Elevated HbF seen in β-thalassemia, HPFH, and some leukemias.
HbF ensures fetal oxygenation; its pharmacologic reactivation is a cornerstone of hemoglobinopathy treatment.