The history of Köln’s hemoglobin starts with a small notification delivered at a Hemoglobin Colloquium in Vienna. Pribilla (1962) reported that an abnormal hemoglobin component had been found on a family from middle Reno, which suffered from a condition similar to thalassemia. They did not have family relations with Mediterranean, African or Asian people. This component, which in electrophoresis formed a smaller band between hemoglobin A and hemoglobin A₂, and which comprised about 5% of total hemoglobin, appeared as a “new” hemoglobin and was called Köln’s hemoglobin. A more detailed description of the case in this family was given by Pribilla and colleagues (1965) with the title Hämoglobin-Köln-Krankheit: familiäre hupocrome hämolytische anämie mit hämoglobinamalie. Of the nine cases found in four generations, five were confirmed and four remained inconclusive. The patients were moderately anemic: hemoglobin 10.5-13.5g/dl, reticulocytes 190-270 x 10⁹/l, MCH 26-28pg, MCV 96-122fl, bilirubin 1.0-2.5mg/dl; there were anisocytosis and basophilic dots. The osmotic fragility were moderately reduced. All had splenomegaly. Physico-chemical studies led to the conclusion that the abnormal hemoglobin was characterized as an abnormality in its b-globin chain between residues 83 and 120. It was later demonstrated by Carrell, Lehmann and Pribilla (1967), in Cambridge, that the German Köln’s hemoglobin had the same abnormality (methionine instead of valine in position b 98) as had been previously found in two families in Great Britain (1).

the term unstable hemoglobins must only be used for those hemoglobins that produce Heinz inclusion bodies in patients with acute or chronic hemolytic anemia

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