Hemoglobin Sabine

Very rare. Hemoglobin abnormality due to a mutation of the HBB gene resulting in the replacement of leucine with proline on the hemoglobin β chain. This mutation leads to hemoglobin fragility with chronic hemolytic anemia, gradually leading to splenomegaly and vesicular lithiasis.


Anesthetic implications: 

check hemoglobin and platelet levels (hypersplenism ?)


References : 

-         Yamamoto K, Nakamura K, Shiozawa R, Iwashima S, Mimaki M.
First report of familial hemolytic anemia due to hemoglobin Sabine in Asia.
Pediatrics International 2022; 64: e15081


Updated: May 2022