Hemoglobin Milwaukee

(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