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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.