Background: Porphyria comprises a group of metabolic disorders caused by the irregular activities of enzymes within the haem biosynthetic pathway. This disease can provoke a large variety of symptoms. Acute porphyria attacks need to be treated urgently to avoid prolonged illness and fatal complications. Haem arginate, a concentrated haem solution stabilized with arginine, is the only preparation available for treatment in Europe and South America. This report describes a safe desensitization protocol for patients who require such treatment and have haem arginate hypersensitivity. Case presentation: A 25-year-old female patient diagnosed with acute intermittent porphyria, who had an anaphylactic reaction while receiving haem arginate. The patient was treated with a desensitization protocol for patients with hypersensitivity to haem arginate. Conclusion: Porphyria is a disease that can significantly compromise a patient's quality of life. The desensitization protocol for patients with hypersensitivity to haem arginate is a safe and effective treatment option for patients with a history of haem arginate allergies, to whom it is not possible to administer haematin. Keywords: Porphyria, Hypersensitivity to haem arginate, Hemin arginate
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