[Diagnosis of hemolytic anemia with unstable hemoglobin]

Heinz-body haemolytic anaemia represents a rarely occurring kind of hereditary defect of haemoglobin, G-6-PDH or glutathion reductase. The course of disease observed in two patients with non-spherocytic haemolytic anaemia was very serious as compared with other cases with haemoglobin variants and enzyme defects of G-6-PDH described in literature. The course of disease could not be influenced by splenectomy, substitution therapy, and long-term therapy with desferrioxamin. Exitus occurred at an age 22 or 41 as a consequence of severe haemosiderosis.