doi:10.1093/qjmed/hch027 Review Managing malignant disease in patients with porphyria

Certain types of porphyria have an increased incidence of malignant disease. In addition, patients with all forms of porphyria may develop malignant disease as a ‘normal life event’. The investigation and treatment of porphyric patients with malignant disease requires specific precautions to minimize the risk of an acute porphyric attack, and to ensure optimum treatment of the malignancy. We briefly review the biochemical basis, clinical features and current management of porphyria in cancer patients. Biochemical basis of the porphyrias The porphyrias represent a collection of seven disorders due to genetic defects in haem biosyn-thesis (Table 1). The pathway starts and finishes in the mitochondrial matrix, so that the final produc