Objectives. To study whether primary liver cancer (PLC) could be assoc
iated with acute intermittent porphyria (AIP) carriership and whether
the activity of erythrocyte porphobilinogen deaminase (PBGD) could be
used as a tumour marker for PLC. Design. Prospective study. Setting. M
edical and surgical wards in two general hospitals in Goteborg, Sweden
. Subjects. All patients with a strong suspicion of PLC (n = 109) who
came to the authors' attention. Main outcome measures. Measurement of
PBGD activity in erythrocytes. Comparison of the PBGD activity in grou
ps with various final diagnoses-hepatocellular carcinoma (n = 58), cho
langiocellular carcinoma (n = 2), malignancy other than PLC (n = 18),
benign liver disorders (n = 11)-and according to presence of cirrhosis
. Results. None of the patients had a clinical or family history of AI
P. Four cases with low PBGD activity, suggesting AIP gene carriership,
were found, which is more than expected. However, the cases were even
ly distributed amongst the groups. The mean activity of PBGD was highe
r in cirrhotic patients, irrespective of the presence of PLC, than in
others. Conclusions. (i) Acute intermittent porphyria gene carriership
might be associated with an increased risk not only for PLC but also
for secondary malignancies and benign tumours in the liver. (ii) High
activity of PBGD is not unusual in liver cirrhosis and the reason for
this needs to be elucidated, but it seems to be of no clinical value a
s a tumour marker for PLC.