Glucose-6-Phosphate Dehydrogenase and Pyruvate Kinase in Sickle Cell Anemia

The glucose-6-phosphate dehydrogenase (G-6-PD) and pyruvate kinase (PK) were determined in sickle cell anemia patients using kits from Boehringer Mannheim. The level was found to be 155±62 mU/109 erythrocytes and 414.3±188 mU/109 erythrocytes, for G-6-PD and PK respectively. These levels were significantly higher than the levels obtained in normal individuals. The enzyme levels were subjected to regression and correlation analysis with the hematological parameters in these patients. A negative correlation was obtained between the enzyme level (G-6-PD, PK) and total hemoglobin, red blood cell count and packed cell volume, while a positive correlation was demonstrated with MCV, MCH and white blood cells. No correlation was demonstrated between these enzymes and MCHC, reticulocytes, Hb A2 and Hb F. These results show that G-6-PD and PK levels are elevated in patients with sickle cell anemia. This may be a consequence of associated elevation in neocytes, or due to a real increase in G-6-PD level or due to a modified phenotypic expression of G-6-PD. Thus an increase in the anemic state may result in an increase in the G-6-PD and PK activity, thereby masking any associated enzyme deficiency, particularly in patients who have a partial enzyme deficiency and when commercially available kits are used.