1986 Volume 27 Issue 9 Pages 1645-1649
A 20 year old woman suffering from hemolytic anemia was admitted to our hospital because of exacerbated hemolysis after EB virus infection. Laboratory data were compatible to unstable hemoglobin hemolytic anemia and cellulose acetate membrane electrophoresis (pH 8.6) showed abnormal band between Hb A and Hb A2. PCMB treatment of hemolysate caused massive yellow-white colored precipitate, most of which was identified to be composed of β-chain by isoelectric focusing. Although tryptic fingerprinting of aminoethylated abnormal β-chain showed no abnormality, amino acid analysis of each peptide extracted from the tryptic fingerprint revealed the substitution of valine to methionine in βTp-11 and identified to Hb Köln or β 98 (FG 5) Val→Met. Functional analysis of abnormal hemoglobin by OEC showed the increased oxygen affinity and the decreased subunit co-operativity. In family study, this hemoglobin was also found in mother, uncle and two cousins, suggesting that it was inherited by autosomal dominant trait. We reported here the tenth case of Hb Köln found in Japan.
