title: [Diagnosis of hemolytic anemia with unstable hemoglobin].
abstract: 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.
mesh_terms: D000328:Adult; D000746:Anemia, Hemolytic, Congenital Nonspherocytic / Q000175:diagnosis* / Q000601:surgery; D004912:Erythrocytes / Q000201:enzymology; D005260:Female; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000175:diagnosis; D006366:Heinz Bodies; D006455:Hemoglobins, Abnormal / Q000032:analysis; D006801:Humans; D013156:Splenectomy
chemical_list: D006455:Hemoglobins, Abnormal
vernacular_title: Zur Diagnostik der hämolytischen Anämie mit instabilem Hämoglobin.