The issue of treating sickle cell disease with drugs that increase hemoglobin oxygen affinity
has come to the fore with the US Food and Drug Administration approval in 2019 of …

The erythrocytes in sickle cell disease have long been known to show decreased oxygen
affinity compared with those from healthy volunteers. 1–4 This property is measured as an …

New treatments directly targeting polymerization of sickle hemoglobin (HbS), the proximate
event in the pathophysiology of sickle cell disease (SCD), are needed to address the severe …

Sickle cell disease (SCD) is the result of homozygous or compound heterozygous
inheritance of mutation in the b-globin gene. The resulting substitution of the hydrophilic …

Sickle hemoglobin (HbS), caused by a point mutation in the β-globin gene of hemoglobin,
polymerizes when deoxygenated. The pathophysiology of sickle cell disease results from …

The accelerating development of potential therapeutic agents for sickle cell anemia (SCA) is
welcome and encouraging. Yet, our present comments are prompted by one of these drugs …

The effect of oxygen therapy on the number of irreversibly (ISC) and reversibly (RSC) sickled
cells was studied in patients with sickle cell anemia. Inhalation of 50% oxygen in patients …

The hallmark of sickle cell disease (SCD) is the polymerization of deoxygenated sickle
hemoglobin (HbS). In SCD patients, one strategy to reduce red blood cell (RBC) sickling is …

Although it has been known for more than 60 years that the cause of sickle cell disease is
polymerization of a hemoglobin mutant, hydroxyurea is the only drug approved for treatment …

Fetal hemoglobin (HbF) inhibits the root cause of sickle pathophysiology, sickle hemoglobin
polymerization. Individuals who naturally express high levels of HbF beyond infancy thus …