treatment
Last reviewed 01/2018
Treatment is largely preventive and symptomatic.
Preventive measures include avoidance of sunlight, monitoring of anaemia, and treatment of recurrent skin infection. Oral administration of charcoal and colestyramine to absorb porphyrins may be helpful (1).
Splenectomy for intractable haemolytic anaemia has successfully improved both the anaemia and the cutaneous sensitivity in some patients.
Suppression of erythropoiesis by transfusion of packed erythrocytes causes a marked decrease in porphyrin production and excretion with concomitant decrease in erythropoiesis, erythropoietic production and plasma iron turnover.
Bone marrow suppression may be achieved with hydroxyurea administration. Allogenic bone marrow transplantation has been successful in treating this condition (1).
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