USMLE (Fach) / Hematology and Oncology (Lektion)
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G6PD deficiency
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Most common enzymatic disorder of RBCs. Causes extravascular and intravascular hemolysis.- X-linked recessive- African, Mediterranean, and Asian descent
Defect in G6PD → ↓ glutathion → RBC susceptibility to oxidant stress.Hemolytic anemia following oxidant stress (eg, sulfa drugs, antimalarials, infections, fava beans).
Findings:- Back pain- Hemoglobinuria a few days after oxidant stress.- Jaundice
Labs: blood smear shows RBCs with Heinz bodies and bite cells (degmacytes)
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