ABSTRACT
Therapeutic strategy Before any treatment is prescribed, potentially eliminative triggering factors for PCT, such as sun light exposure, estrogen intake, alcohol abuse, ingestion of chlorinated hydrocarbons (hexachlorobenzene), and iron overload, should be sought, with tests for HIV and HCV infections if indicated. Phlebotomy therapy has been the mainstay of treatment for some time. In a previous study, blood removal of 2500-4500 cc over a 3-to 4.5-month period showed a marked reduction of urine and fecal porphyrin secretions in most patients, with accompanying clinical improvement [3]. The usual schedule for phlebotomy is a weekly or biweekly withdrawal of 500 cc until the hemoglobin level reaches 10 g/dl. The recommended treatment is now chloroquine-class medications, with phlebotomy only for PCT patients with iron overload and HCV infection [9]. Low-dose chloroquine (125 mg twice/week for 8-18 months) has been very useful. A combination of phlebotomy and chloroquine has been suggested for some patients, for the purpose of reducing hepatotoxicity and accelerating remission. A high dose of vitamin E, a known antioxidant, has recently been shown to reduce urine porphyrin levels [7].
