Hemoglobin Sabine
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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