Group of rare abnormalities of the hemoglobin. Most are little or even not symptomatic. Some lead to chronic hemolytic anemia. From the anesthetic point of view, some lead to desaturation (or measured with SpO2) poorly or not corrected by the administration of O2.

The possible and sometimes combined mechanisms are:

-        a falsely low SpO2 measurement: Hb Bonn, Hb Cheverly, HbM Iwate, Hb Köln, Hb Lansing

-        the presence of methemoglobin: HbM Boston, Hb Chile, HbF Fort Ripley, Hb Freiburg, HbM Hydepark, HbM Iwate, HbM Milwaukee, HbFM Osaka, HbM Saskatoon, Hb St Louis

-        a decreased affinity of hemoglobin for O2 : Hb Arta, Hb Bassett, Hb Beth Israel, Hb Canebiere, Hb Cheverly, Hb Chico, Hb Hammersmith, Hb Hazebrouck, Hb Hope, Hb Kansas, Hb Kirksey, Hb Louisville, Hb Nishinomiya, Hb Rothschild, Hb Seattle, Hb St Mande, Hb Sunshine Seth, Hb Titusville, Hb Venusberg, Hb Warsaw, Hb Yoshizuka.

Site to visit to know all the characteristics of any variant of human hemoglobin.

http://globin.cse.psu.edu

Anesthetic implications: 

confirm the measurement of SpO2 by transcutaneous paO2 or blood gases. The importance of the desaturation, real or fake, also depends on the % of abnormal Hb present in the blood.

References:

•        Zur B, Ludwig M, Stoffel-Wagner B. 
  Case-control studies of novel hemoglobin anomalies as differential diagnosis in sleep apnea syndrome.
  Sleep Breath 2013; 17: 557-63

Updated: July 2019