G6PD Deficiency vs Warm Autoimmune Hemolytic Anemia
G6PD Deficiency and Warm Autoimmune Hemolytic Anemia are easy to mix up on the boards. Here's a side-by-side comparison — presentation, workup, imaging, and first-line treatment — drawn from our full outlines.
G6PD Deficiency vs Warm Autoimmune Hemolytic Anemia at a glance
- G6PD Deficiency: X-linked enzyme deficiency causing episodic oxidative hemolysis in response to drugs, infection, or fava beans.
- Warm Autoimmune Hemolytic Anemia: IgG-mediated extravascular hemolysis with positive direct Coombs (DAT) — first-line treatment is steroids.
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Side-by-side comparison
| Feature | G6PD Deficiency | Warm Autoimmune Hemolytic Anemia |
|---|---|---|
| At a glance | X-linked enzyme deficiency causing episodic oxidative hemolysis in response to drugs, infection, or fava beans. | IgG-mediated extravascular hemolysis with positive direct Coombs (DAT) — first-line treatment is steroids. |
| Classic presentation | Dark urine and jaundice 1-3 days after starting TMP-SMX (or dapsone, or fava bean ingestion) in a young man of African or Mediterranean descent.; Acute hemolytic episode 24-72 hours after oxidant exposure: dark/cola-colored urine (hemoglobinuria), back/abdominal pain, jaundice, fatigue, dyspnea; Neonatal jaundice — especially… | Anemia with spherocytes on smear and a positive direct Coombs (DAT) for IgG ± C3.; Insidious or acute fatigue, dyspnea on exertion, pallor; Jaundice, dark urine in severe cases; Constitutional symptoms if underlying lymphoma or autoimmune disease; Fever, abdominal pain in fulminant hemolysis; Pallor with scleral icterus; Splenomegaly… |
| Workup / key labs | Low quantitative G6PD enzyme activity in a stable patient (away from acute hemolytic episode). Newborn screening available in many states.; CBC — normocytic anemia, reticulocytosis (peaks ~7 days after trigger); Peripheral smear — bite cells (membrane removed by splenic macrophages around denatured Hb), blister cells, Heinz bodies… | Evidence of hemolysis (elevated reticulocytes, LDH, indirect bilirubin; low haptoglobin) + positive direct Coombs (DAT) for IgG ± C3 + spherocytes on smear.; CBC — normocytic or macrocytic anemia (macrocytic due to reticulocytosis); often other cytopenias if Evans syndrome; Peripheral smear — spherocytes (signature finding from partial… |
| Imaging | Not routinely indicated | CT chest/abdomen/pelvis if lymphoma or solid tumor suspected |
| First-line treatment | Acute hemolysis: identify and remove offending agent immediately; Supportive care — IV fluids to maintain renal perfusion and clear hemoglobinuria; Transfusion for severe symptomatic anemia or hemodynamic instability; Prevention is primary: educate patient about drugs and foods to avoid; provide list of contraindicated medications;… | Prednisone 1 mg/kg/day (typically 60-100 mg) — first-line; ~70-80% initial response; taper slowly over 3-6 months once Hb stabilizes; Folic acid 1-5 mg/day (chronic hemolysis depletes folate); Identify and treat underlying cause: discontinue offending drug, treat lymphoma/CLL, manage autoimmune disease; Transfuse only for… |
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