Hepatic Porphyria

Hepatic Porphyria

Signs and Symptoms
Diagnosis
Treatment

Treatment

Treatment for hepatic porphyria depends on the type. However, for both cutaneous and acute porphyria, the first step of therapy involves identifying and eliminating the precipitating factors, including alcohol, certain medication, excess iron, etc.

• Cutaneous Porphyria -- For patients with cutaneous porphyria whose iron levels are high, iron depletion is recommended. This involves removing the excess iron from the body by drawing your blood, usually a pint at a time in regular intervals. This is done in the same way in which blood is drawn from donors at blood banks. When the body replenishes the blood, it draws iron from storage sites such as the liver. Repeated over time, this process eventually eliminates the excess iron and leads to reduced porphyrin levels and improved sun tolerance.

• Acute Porphyria -- The best form of treatment for acute porphyria is prevention. Therefore, your family members should be tested, to identify those who are also at risk of having an acute attack. When acute porphyria progresses despite elimination of triggering factors, hospitalization is necessary. Specific treatment involves administration of intravenous hematin, which compensates for the genetic deficiency.

Reviewed by health care specialists at UCSF Medical Center.
Last updated May 8, 2007