PCT

Treatment

It is important to discontinue use of alcohol and, if possible, other contributing factors such as estrogens. Resumption of heavy alcohol intake is likely to lead to recurrence. However, in postmenopausal women who have been treated for PCT, there is seldom a recurrence when estrogens are restarted.

The most widely preferred treatment is repeated phlebotomy (venesection). The objective of the treatment is to decrease the amount of iron in the body to the lower limit of normal. Red blood cells contain large amounts of iron (in hemoglobin). When red blood cells are removed from the body, new cells and hemoglobin are made by the bone marrow. With repeated phlebotomy, the body's iron stores are gradually depleted. This process also removes iron from the liver, and the activity of the UROD is gradually restored. A pint of blood is removed every 1-2 weeks until the ferritin reaches the lower limit of normal.

The course of treatment is best followed by measuring the serum ferritin and plasma or serum porphyrin levels. Blood hematocrit or hemoglobin is also measured to avoid developing significant anemia. The plasma porphyrin levels also then gradually decrease, and the development of new skin lesions stops. Healing of damaged skin occurs more gradually. After the plasma porphyrin levels become normal, the patient is able to tolerate sunlight.

Low-dose chloroquine is a suitable alternative treatment, especially in patients who cannot tolerate phlebotomies. Chloroquine mobilizes excess porphyrins from the liver. Normal doses of chloroquine (as used for treating malaria or rheumatoid arthritis) can markedly worsen PCT and also cause liver damage before inducing a remission of PCT. If very small doses are used for treating PCT, these adverse effects are usually avoided. Only 125mg of chloroquine or 100mg of hydroxychloroquine should be given to patients twice weekly. Because tablets this small are not manufactured, the tablets must be cut by the patient or a pharmacist. Chloroquine and hydroxychloroquine rarely damage the retina of the eye.

PCT may be unusually severe in patients with advanced kidney diseases. Erythropoietin and phlebotomy, but not low-dose chloroquine, can be effective in these cases.

Treatment of hepatitis C with interferon alpha and ribavirin is available but is often not effective. Patients with PCT and hepatitis C should usually first undergo treatment of PCT. Treatment of hepatitis C can be considered later. There is some evidence that iron depletion may improve the results of treatment of hepatitis C.

The treatment of PCT is almost always successful, and the prognosis is usually excellent. The condition is not progressive and is seldom disabling. After treatment, periodic measurement of plasma porphyrins may be advised, especially if a contributing factor such as estrogen exposure is resumed. If a recurrence does occur, it can be detected early and retreated promptly.