Hemoglobin Southampton

Very rare. Abnormal hemoglobin due to a mutation  in position 106 in the ß chain where a proline is replaced by a leucine. This results in an instability of hemoglobin and a falsely low measure of its saturation level.

Chronic hemolysis that requires repeated transfusions; vesicular lithiasis, risk of iron overload.


Anesthetic implications:

check the hemoglobin level; be aware that pulseoxymetry values are falsely low at room air


References : 

-        Costandi AJ, Mahmoud M. 
Unexpected falsely low pulse oximeter measurements in a child with hemoglobin Southampton.
J Clin Anesth 2016; 33: 320-1


Updated: September 2016