Haem arginate

How it works

It replenishes the regulatory haem pool, suppressing aminolevulinic acid synthase activity and thereby reducing overproduction of porphyrin precursors that drive an acute attack.

Prescribing in practice

• It must be given by intravenous infusion into a large vein because of the risk of thrombophlebitis and extravasation injury.
• Treatment should begin early in an acute attack alongside supportive care and removal of precipitating factors.
• It should be used under specialist guidance for confirmed acute porphyria.

Monitoring

Monitor the infusion site, clinical symptoms of the attack and relevant biochemistry to assess response.

Counselling the patient

• The infusion is given into a large vein to reduce vein irritation.
• Avoiding known trigger factors and medicines helps prevent future attacks.
• Tell clinicians you have porphyria before any new medicine is started.

Evidence & guidelines

Intravenous haem replacement is the established treatment for acute attacks of hepatic porphyria in specialist practice.