You confirm your diagnosis and begin treatment which is shown above (given as an IV bolus of 1-2mg/kg over 5 minutes). The patient does not get better after a few hours. In fact, he seems to get worse; he's more dyspneic. You decide to send another panel of labs, and when they come back, you note that the hemoglobin has dropped 3 points and the blood smear shows:
Challenge: What happened?
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hemolysis 2/2 to methemoglobinemia--concern for G6PD or Pyruvate kinase deficiency
nice!! i thought this was pretty tricky
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Hurricane II
The treatment for methemoglobinemia is methylene blue to help reduce methemoglobin by an NADPH-dependent pathway. However, this is contraindicated if the patient has glucose-6-phosphate dehydrogenase deficiency since this pathway dependents on G6PD to generate NADPH. In this case, methylene blue can trigger hemolysis. The smear shows characteristic bite-like deformities on the left and denatured hemoglobin precipitates on the right.
Sources: UpToDate; Wikipedia.
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