ABSTRACT
Sickle cell disease is a hereditary blood disorder characterized by chronic manifestations of progressive organ failure. To control a disease of this type, it is important to study the factors which affect the course of the disease. Diphosphoglycerate [2, 3 DPG] is the most common metabolite generated by the glycolysis. It is a potent modifier for hemoglobin function. To clarify the role of 2, 3 DPG in sickling, its concentration was measured in 15 normal healthy individuals and in 12 sickle cell patients at steady state and during crisis. Hematological indices and partial oxygen tension [PO[2]] were also determined for sicklers and controls. Sickle cell patients during crisis were found to have a significantly higher levels of 2, 3 DPG [9.17 +/- 5.36 mmol/L] [p<0.05], and significantly low PO[2] [8.41 +/- 1.82 Kpa, p< 0.01] than controls [8.54 +/- 0.72 mmol/L for 2, 3 DPG and 12.44 +/- 0.7 Kpa for PO[4]]. The increase in 2, 3 DPG concentration during crisis may be due to its increased production via glycolysis, which in turn increased as a result of decreased oxygen tension during crisis. The increased 2, 3 DPG concentration increases and stabilizes the deoxygenated HbS leading to its precipitation, sickling, and hemolysis of red blood cells. In conclusion, the factors which can increase 2, 3 DPG or decrease PO[4] may lead to the sickling crisis and increase the phenotypic severity of the disease