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Hematology
hemolytic anemia
g6pd deficiency
A 25-year-old female presents to the emergency department complaining of sudden onset of dark urine, profound fatigue, and yellowish discoloration of her eyes and skin, which began yesterday. She reports having consumed a traditional Mediterranean dish containing fava beans during a family gathering two days prior. Her medical history is otherwise unremarkable, and she denies any recent infections. On physical examination, she appears pale and jaundiced. Her heart rate is 105 bpm, blood pressure is 110/70 mmHg, and she is afebrile. Abdominal examination reveals mild splenomegaly, with a palpable spleen tip 2 cm below the left costal margin. There are no signs of hepatomegaly or ascites. Peripheral blood smear shows numerous fragmented red blood cells, anisopoikilocytosis, and a significant number of cells with membrane irregularities and precipitated hemoglobin inclusions. What is the most likely diagnosis and the immediate priority for her management?
| Lab Parameter | Value | Reference Range |
|---|---|---|
| Hemoglobin | 85 g/L | 120-150 g/L |
| Hematocrit | 0.25 L/L | 0.36-0.44 L/L |
| Reticulocyte Count | 0.08 (8%) | 0.005-0.015 (0.5-1.5%) |
| Total Bilirubin | 55 µmol/L | 5-21 µmol/L |
| Indirect Bilirubin | 48 µmol/L | <17 µmol/L |
| Lactate Dehydrogenase (LDH) | 450 U/L | 140-280 U/L |
| Haptoglobin | <0.1 g/L | 0.4-2.0 g/L |
| Urinalysis | Positive for blood, no red blood cells | Negative for blood |
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