(see also Hereditary Methemoglobinemias)

(Hemoglobinosis M-Milwaukee-2, Hemoglobinosis M-Akita)

Rare. Dominant autosomal transmission of a mutation on the  β chain where tyrosine replaces histidine. This mutation results in a constant methemoglobinemia of about 10 % and a decreased affinity for oxygen .

Anesthetic implications: 

cyanosis, falsely elevated pulseoxymetry values at room air, not responding to oxygen therapy; methemoglobinemia unresponsive to the administration of methylene blue

References : 

-        Picca A, Ruthford M, Ghanim MT, Sims M, Kanter J.
Diagnosis of hemoglobin M Disease in a toddler presenting with hypoxemia and hemolysis.
Clin Pediatr 2019; 58: 1345-8.

Updated: November 2019