Summary
We report a fourth case of Hb Nottingham [α2β2 98 (FG5) Val→Gly] observed in an 8-year-old girl in the Czech Republic with clinical and laboratory symptoms of severe hemolytic anemia. The unstable hemoglobin probably represents a de novo mutation, since the parents of the patient and the two siblings do not exhibit any hematological abnormalities. Splenectomy had a beneficial effect on the degree of hemolysis, as well as on the Hb level.
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Brabec, V., Indrák, K., Fořtová, H. et al. Hb Nottingham or α2β2 98 (FG5) Val→Gly in a Czech child. Ann Hematol 69, 93–95 (1994). https://doi.org/10.1007/BF01698489
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DOI: https://doi.org/10.1007/BF01698489