Iodination Defect in the Leukocytes of a Patient with Chronic Granulomatous Disease of Childhood

Abstract

In a case of chronic granulomatous disease, the patient's neutrophils, unlike normal neutrophils, did not fix iodine after ingestion of heat-killed Lactobacillus acidophilus or heat-killed or live Serratia marcescens. When live L. acidophilus was employed, iodination did occur, although it was less than normal. The patient's mother had some neutrophils that did not and some neutrophils that did iodinate after ingestion of heat-killed L. acidophilus. Unlike S. marcescens, L. acidophilus was killed as well by the patient's leukocytes as by normal leukocytes. Methimazole inhibited the killing of L. acidophilus by the leukocytes of the patient and the patient's mother but had no significant effect when normal leukocytes were employed. The ability of leukocytes in chronic granulomatous disease to kill certain organisms may depend on bacterial formation of the hydrogen peroxide required for an antimicrobial system in the leukocytes, mediated by myeloperoxidase.