THE MANAGEMENT OF PORPHYRIC PATIENTS WITH MALIGNANT DISEASE: A REVIEW OF THE LITERATURE AND RECOMMENDATIONS FOR THE FUTURE

Carlo Palmieri1, David M.Vigushin1, Timothy J Peters2

1Department of Cancer Medicine, Faculty of Medicine, Imperial College, London and 2Department of Clinical Biochemistry, King's College Hospital, London, UK.

Porphyria is a collection of seven disorders caused by genetic defects in the biosynthesis of haem which clinically can be subdivided into the neuropsychiatric (acute), dermatological or mixed types. The mainstay of the treatment of porphyria is the prevention of acute attacks and the avoidance of skin lesions. The avoidance of porphyric drugs is a key part of this prevention strategy. It is known that certain types of porphyrias have an increased risk of malignant disease such as hepatocellular carcinoma in acute intermittent porphyria and porphyria cutanea tarda. In addition, an acute porphyric attack may be the manifestation of an underlying malignancy. Porphyric patients like any member of the general population may develop a malignant disease. The investigation and management of such porphyric patients requires particular care and attention to ensure that an acute attack is not precipitated, and that the patients receive optimal care and attention for their underlying malignancy. In addition treating units must be aware of the signs and symptoms of an acute attack and its management. We reviewed all the available English literature relating to the treatment of malignant disease in porphyric patients. Based on this literature the investigation of porphyric patients who may have an underlying malignant disease, as well as the treatment of such patients will be discussed. Drugs used for symptomatic treatment and in the palliative setting will also be reviewed. We have developed the Imperial College London-King’s College Hospital guidelines for the management of oncology patients with a history of porphyria. It is hoped that these recommendations will help to improve the care and outcome of such porphyric patients.