Hungarian hereditary acatalasemia and hypocatalasemia are not associated with chronic hemolysis.

Two Hungarian acatalasemic and eight hypocatalasemic patients revealed normal erythropoesis. Contrary to their decreased defence system against the toxic hydrogen peroxide, the biochemical tests (serum catalase, serum hemoglobin, serum lactate dehydrogenase (LDH) ratio of LDH1 and LDH2 isoenzymes and serum haptoglobin) excluded hemolysis. The normal activity of glutathione peroxidase and the decreased catalase activity could prevent the lysis of the erythrocytes. In the presence of extremely high levels of hydrogen peroxide acute hemolysis may not be excluded; therefore, follow-up of these patients is required.