Sickle Cell Disease and Inositol Phosphates
Pp. 176-186 (11)
A.K.M. Shamsuddin and Guang-Yu Yang
Sickle cell disease (SCD) is a hereditary blood disorder due to a mutation in
the hemoglobin gene, and characterized by abnormal hemoglobin S (HbS). Under
hypoxic conditions, HbS polymerizes, inducing distortion of RBCs into rigid sickleshaped
cells; in particular, homozygous SCD patients (SS) may experience painful
episodes and complications due to vaso-occlusion (VOC) during their lifetime. There is
no cure, but symptomatic management to improve anemia and lower the complications
through blood transfusions is currently the most accepted therapy for those patients.
Chronic red blood cell (RBC) exchanges or transfusion are effective in preventing the
recurrence of VOC. While simple blood transfusions are important therapy for SCD, the
goal of efficient transfusion is to increase the oxygen delivery and to prevent the
sickling. It has been demonstrated that InsP6-loaded red blood cells (InsP6-RBCs)
reduce the risks of sickling of sickle RBCs (SS RBCs) exposed to hypoxia.
InsP6, InsP6-loaded red blood cells, malaria, oxy-hemoglobin
dissociation curve, sickle cell anemia.
The University of Maryland School of Medicine 10 S. Pine Street, MSTF-700 Baltimore, MD 21201-1116 USA., Northwestern University Feinberg School of Medicine 303 E Chicago Ave, Ward 4-115 Chicago, IL 60611 USA.