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Methemoglobinemia
Guidelines
Key sources
The following summarized guidelines for the evaluation and management of methemoglobinemia are prepared by our editorial team based on guidelines from the American Heart Association (AHA 2023) and the European Reference Network on Rare Hematological Diseases (ERN-EuroBloodNet/EHA 2021).
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Screening and diagnosis
Diagnostic investigations
Clinical assessment
As per EHA/ERN-EuroBloodNet 2021 guidelines:
Assess clinical findings and elicit family history to distinguish acquired from inherited forms of methemoglobinemia.
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Recognize the neurologic and cardiac symptoms and their progression with increasing methemoglobin.
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Methemoglobin levels
Testing for NADH-cytochrome b5 reductase deficiency
Testing for G6PD deficiency
Respiratory support
Oxygen supplementation: as per EHA/ERN-EuroBloodNet 2021 guidelines, administer oxygen supplementation if needed in minimally symptomatic or asymptomatic patients. Obtain routine monitoring of oxygen saturation with pulse oximetry in case oxygen is initiated, at least in neonates.
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Hyperbaric oxygen therapy
Medical management
General principles: as per EHA/ERN-EuroBloodNet 2021 guidelines, manage methemoglobinemia in pediatric patients based on the symptoms, level of methemoglobin, cause of methemoglobinemia, and patient's age.
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Methylene blue
Ascorbic acid
N-acetylcysteine
Therapeutic procedures
Exchange transfusion: as per AHA 2023 guidelines, consider performing exchange transfusion in patients with methemoglobinemia not responding to methylene blue.
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Therapeutic phlebotomy