Hemoglobin Köln
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The most frequent form of unstable hemoglobin. Autosomal dominant transmission of the mutation 98 (FG5) Val à Met of the chain ß of the hemoglobin.
This mutation results in:
- instability of heme (Hb precipitates in red blood cells where it forms Heinz bodies) with chronic hemolysis: mild jaundice, vesicular lithiasis, splenomegaly
- acute hemolysis can occur during an infection or the administration of sulfonamides
- increased affinity of hemoglobin for O2 : P50 = 17.6 mmHg, and therefore chronic relative tissue hypoxia; in addition, a modified light absorption by Hb Köln causes a false low reading of SpO2
- increased risk of thrombosis after splenectomy: the high erythropoietin concentration due to tissue hypoxia increases platelets count.
Anesthetic implications:
complete blood count; falsely low pulse oximetry values with small response to FiO2 increase; if in doubt, make a sample of blood gases to measure PaO2.
References :
- Katoh R, Miyake T, Arai T.
Unexpected low pulse oximeter readings in a boy with unstable hemoglogobin Köln.
Anesthesiology 1994; 80: 472-4
- Gottschalk A, Silverberg M.
An unexptected finding with pulse oximetry in a patient with haemoglobin Köln.
Anesthesiology 1994; 80: 474-6
- Andrieu V, Dumonceau O, Grange M-J.
Priapism in a patient with unstable hemoglobin : hemoglobin Köln.
Am J Hematol 2003; 74: 73-4.
- Brugger S, Santafé-Marti MD, Lakhal M.
Low SpO2 with normal SaO2 during general anesthesia: a case report.
A&A Case Reports 2017; 9: 197-7.
Updated: October 2017