Abstract
A 7-year-old girl with Hb Köln, who showed mild hepatosplenomegaly and severe anemia (RBC 216 × 104/mm3, Hb 5.2 g/dl, Ht 19.1%, reticulocyte 1%0) associated with an apparently upper respiratory infection, was reported. Erythroblast hyperplasia was observed in bone marrow. Further examination of hemoglobin revealed an abnormal band between Hb A and Hb A2 in cellulose acetate membrane electrophoresis (pH 8.6). In the analysis of β chain separated by reverse-phase high-performance liquid chromatography, a change of valine to methionine, consistent with Hb Köln, was recognized. No other member of her family (father, mother, an elder brother and an elder sister) had the abnormal hemoglobin.
