Functional Analysis of Polymorphonuclear Leukocytes in Siblings of Glucose-6-Phosphate Dehydrogenase Deficiency

Several functions of polymorphonuclear leukocytes (PMNL) were examined in two siblings with glucose-6-phosphate dehydrogenase (G6PD) deficiency. In spite of marked depression of G6PD activity of PMNL, the patients had no susceptibility to bacterial infections. Qualitative nitroblue tetrazolium (NET) reduction was normal in both cases. Quantitative NBT reduction was normal in one case and subnormal in the other. Bactericidal activity also was normal. However, superoxide anion (O2-) release, oxygen (O2) consumption and chemiluminescence (CL) response were significantly decreased in both cases. This dysfunction was more marked when glucose was added extracellularly. It is concluded that these tests are more suitable than the NBT test for the screening of PMNL G6PD deficiency. It is also considered that there is a large reserve supply of active oxygens necessary for normal bactericidal activity of PMNL.
This work has been supported in part by Grants-in-Aid for Scientific Research (Project Nos. 544050, 448227) from the Ministry of Education, Scientific and Culture of Japan and a Grant-in-Aid from the Japan Medical Research Foundation.