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Three unrelated Japanese patients with chronic nonspherocytic hemolytic anemia were found to have marked deficiency of red blood cell (RBC) reduced glutathione (GSH) (4.4%, 13.1%, and 6.9% of normal, respectively). A panel of RBC enzyme assays showed that one patient had decreased glutathione synthetase activity and the other two were moderately deficient in γ-glutamylcysteine synthetase. Some family members of each patient showed mild deficiency of the respective enzymes. RBCs of these patients also showed a decreased level of glutathione-S-transferase as in previously described GSH-deficient cases. Hemolytic anemia was their only manifestation, and neither 5-oxoproli-nemia nor 5-oxoprolinuria, which are usually associated with the generalized type of glutathione synthetase deficiency, was noted in our patients.