Hemoglobin Milwaukee
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(see also hereditary methemoglobinemias)
Very rare. Autosomal dominant transmission of a mutation in the β chain in position 67 where a glutamine replaces a valine. This mutation causes permanent methemoglobinemia (15-30 %) and a decreased affinity of hemoglobin for the O2 .
Anesthetic implications:
cyanosis, falsely high pulse oximetry values in room air; methemoglobinemia, which does not respond to the administration of methylene blue
References :
- Stucke AG, Riess ML, Connolly LA.
Hemoglobin M (Milwaukee) affects arterial oxygen saturation and makes pulse oximetry unreliable.
Anesthesiology 2006; 104: 887-8.
Updated: August 2019