Very rare: one case described (China). Abnormality of hemoglobin due to the replacement of aspartic acid by histidine at position 95 on the α chain of hemoglobin. This abnormality leads to a decrease in the affinity of the mutated hemoglobin for O2, responding to the increased FiO2.

Anesthetic implications:

falsely low measurement of SpO2  (pulse oximetry) at room air; monitoring by transcutaneous paO2 or blood gases.

References :

-        Geng ZY, Xue C, Guo JF.
Low oxygen saturation measured by pulseoximetry with a novel haemoglobin variant, Haemoglobin PKU.
Eur J Anaesthesiol 2021 ; 38:1106-6.

Updated: October 2021