Deficiency of Glutathione Peroxidase Associated with High Levels of Reduced Glutathione in Glanzmann's Thrombasthenia

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Abstract
Six patients with documented defects in platelet function were screened for possible abnormalities in platelet energy metabolism and reductive capacity. Abnormalities found in the reductive capacity of three unrelated patients with Glanzmann's thrombasthenia consisted of markedly decreased glutathione peroxidase activity in all three, and were associated with raised levels of reduced glutathione in two of them. Lactate production (glycogenolysis and glycolysis) and adenosine triphosphate levels were normal in the one patient with Glanzmann's thrombasthenia in whom this could be measured.