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Table 2 Annual incidence rates and hazard ratios for neoplasms in persons with porphyria cutanea tarda (2000–2016, 18 years or older)

From: Porphyria cutanea tarda increases risk of hepatocellular carcinoma and premature death: a nationwide cohort study

Cancer codes (ICD-7; ICD-10) Cancer cases/no. at risk Person-years; annual incidence of cancer diagnosis per 100 (95% CI) Hazard ratios (95% confidence intervals) E-value
Crude Adjusted
All sites – first primary C (140–207; C00–96, D45–47)
 Reference 358,701/5451951 70,185,262; 0.507 (0.506–0.509) 1.0 1.0 1.0
 PCT (total) 80/589 8368; 0.956 (0.768–1.190) 1.1 (0.9–1.4) 1.1 (0.9–1.3) 1.4
  Familial PCT 25/245 3728; 0.671 (0.453–0.992) 0.9 (0.6–1.4) 0.9 (0.6–1.3) 1.0
  Sporadic PCT 34/243 3547; 0.959 (0.685–1.342) 1.1 (0.8–1.5) 1.0 (0.7–1.4) 1.1
  Unclassified PCT 21/101 1094; 1.920 (1.252–2.945) 1.8 (1.1–2.7) 1.6 (1.0–2.5) 2.6
All sites – second primary C (140–207; C00–96, D45–47)
 Reference 57,505/5451951 71,305,121; 0.081 (0.080–0.081) 1.0 1.0 1.0
 PCT (total) 26/589 8424; 0.309 (0.210–0.453) 2.4 (1.6–3.5) 2.2 (1.6–3.3) 3.8
  Familial PCT 6/245 3749; 0.160 (0.72–0.356) 1.5 (0.7–3.3) 1.4 (0.6–3.1) 2.2
  Sporadic PCT 14/243 3557; 0.394 (0.233–0.665) 2.9 (1.7–4.9) 2.8 (1.7–4.7) 5.0
  Unclassified PCT 6/101 1119; 0.536 (0.241–1.194) 2.8 (1.2–6.1) 2.5 (1.1–5.6) 4.4
Colon/rectum (153–154; C18-C21)
 Reference 46,077/5451951 71,375,873; 0.503 (0.501–0.504) 1.0 1.0 1.0
 PCT 11/578 8564; 0.936 (0.752–1.165) 1.2 (1.0–1.5) 1.1 (0.9–1.4) 1.4
Hepatocellular carcinoma (155.0; C22.0)
 Reference 1543/5451951 71,538,150; 0.002 (0.002–0.002) 1.0 1.0 1.0
 PCT (total) 6/589 8567; 0.070 (0.031–0.156) 19.9 (8.9–44.3) 19.7 (8.8–44.0) 38.9
Gallbladder and biliary tract (156; C23-C24)
 Reference 1628/5451951 71,537,477; 0.003 (0.003–0.003) 1.0 1.0 1.0
 PCT 3/589 8576; 0.035 (0.011–0.108) 7.2 (2.3–22.5) 6.8 (2.2–21.0) 13.0
Pancreas (157, C25)
 Reference 9830/5451951 71,375,873; 0.014 (0.014–0.014) 1.0 1.0 1.0
 PCT 3/589 8546; 0.035 (0.011–0.109) 1.6 (0.5–4.9) 1.5 (0.5–4.6) 2.4
Lung (162, C33–34)
 Reference 34,920/5451951 71,498,290; 0.049 (0.048–0.049) 1.0 1.0 1.0
 PCT 13/589 8571; 0.152 (0.088–0.261) 2.9 (1.7–5.0) 1.6 (0.9–2.8) 2.6
Non-melanoma skin (191, C44)
 Reference 16,048/5452,951 66,463,540; 0.024 (0.024–0.0024) 1.0 1.0 1.0
 PCT 4/589 8533; 0.047 (0.018–0.125) 1.9 (0.7–5.1) 1.1 (0.4–3.5) 1.4
Breast (170; C50)
 Reference 39,581/2713314 35,414,769; 0.112 (0.111–0.113) 1.0 1.0 1.0
 PCT 5/299 4337; 0115 (0.048–0.277) 0.6 (0.3–1.5) 0.6 (0.3–1.7) 1.0
Prostate (177; C61)
 Reference 56,652/2738637 35,644,567; 0.158 (0.157–0.159) 1.0 1.0 1.0
 PCT 14/290 4170; 0.336 (0.199–0.567) 1.2 (0.7–2.0) 1.1 (0.6–1.8) 1.4
Leukaemia (207; C91–95, D45–47)
 Reference 16,349/5451951 72,014,044; 0.023 (0.022–0.023) 1.0 1.0 1.0
 PCT 4/589 8560; 0.023 (0.018–0.125) 1.4 (0.5–3.7) 1.3 (0.5–3.5) 1.1
  1. Crude analysis adjusted for age as the time-scale. Adjusted analysis covariates include age as time-scale, sex (except for gender specific cancers), educational obtainment, and birth cohorts.
  2. Note that 23 persons diagnosed with cancer prior to their first reported PCT symptoms or diagnosis were excluded. Diagnosis based on the International Classification of Diseases, 7th (ICD-7) and 10th revision (ICD-10) codes.