1968 Volume 9 Issue 4 Pages 545-551
The common type of G6PD deficiency hemolytic anemia usually manifests their symptome immediatry after taking a certain drugs or fava bean.
The present case is 9 year old Japanese boy hemizygote with G6PD deficiency congenital nonspherocytic hemolytic anemia that is a rare type of manifestation of G6PD deficiency.
Each of hemolytic episodes is apparently induced by an acute infection. The activity of G6PD of the red cell of propositus is O unit/100ml erythrocytes in contrast with normal value of 188±25 units/100ml erythrocytes measured by a modified Korberg-Horecker's method, and percentage of ghost cell accounts for 86% comparing with normal value of 0.1±0.1% by the methemoglobin reduction test. Starch gel elctrophoresis of hemolysates of the patient suggested that his G6PD was type B.
Three female heterozygotes were detected from family members by methemoglobin reduction test with intermediately high frequency of ghost cells but no clinical or hematological abnormalities in those family members were noted.
Histochemical methemoglobin reduction test was found to be an useful and reliable procedure for the detection of the carrier state.
