[A new case of hemoglobin G Georgia (author's transl)]

A new case of hemoglobin G Georgia (alpha95 Pro yields Leu) is described in a portuguese patient with lymph node tuberculosis. The patient had a slight anaemia with haemolytic crises and moderate splenomegaly. The electrophoretic mobility of this unstable, abnormal haemoglobin was slower than that of Hb S. The percentage of the major (alpha2 G beta2) and the minor (alpha2 G delta2) abnormal components was determined by DEAE-sephadex chromatography and was 18% and 1% respectively. Structural analysis showed the replacement of a proline residue in position 95 (G) in the alpha chain by a leucine residue. This residue alpha 95 interferes with the alpha 1-bêta 2 contact. Functional studies have shown an increased oxygen affinity, a reduced hem-hem interaction and a normal Bohr effect.