Site-specific and overall risk of cancer for the major PID classifications
| PID classification/cancer site . | O . | E . | SIR (95% CI) . |
|---|---|---|---|
| Predominantly antibody deficiencies, n = 881 | |||
| All malignant neoplasms* | 53 | 32.9 | 1.61 (1.21-2.11)† |
| NHL‡ | 13 | 1.60 | 8.13 (4.33-13.9)† |
| Leukemia | 3 | 0.67 | 4.50 (0.93-13.2) |
| Complement deficiencies, n = 78 | |||
| All malignant neoplasms* | 1 | 2.13 | 0.47 (0.01-2.62) |
| NHL§ | 1 | 0.11 | 9.42 (0.24-52.5) |
| Leukemia | 0 | 0.04 | 0-92.3¶ |
| Combined T-cell and B-cell immunodeficiencies, n = 54 | |||
| All malignant neoplasms* | 0 | 0.32 | 0-11.7¶ |
| NHL | 0 | 0.03 | 0-117¶ |
| Leukemia | 0 | 0.01 | 0-395¶ |
| Other well-defined immunodeficiency syndromes, n = 66 | |||
| All malignant neoplasms* | 3 | 0.23 | 13.0 (2.69-38.1)† |
| NHL‖ | 2 | 0.03 | 62.1 (7.51-224)† |
| Leukemia | 0 | 0.01 | 0-376¶ |
| Congenital defects of phagocyte number, function or both, n = 39 | |||
| All malignant neoplasms* | 0 | 0.48 | 0-7.69¶ |
| NHL | 0 | 0.03 | 0-114¶ |
| Leukemia | 0 | 0.02 | 0-227¶ |
| Diseases of immune dysregulation, n = 14 | |||
| All malignant neoplasms* | 1 | 0.23 | 4.40 (0.11-24.5) |
| NHL | 0 | 0.01 | 0-281¶ |
| Leukemia | 1 | 0.004 | 238 (6.03-1326)† |
| PID classification/cancer site . | O . | E . | SIR (95% CI) . |
|---|---|---|---|
| Predominantly antibody deficiencies, n = 881 | |||
| All malignant neoplasms* | 53 | 32.9 | 1.61 (1.21-2.11)† |
| NHL‡ | 13 | 1.60 | 8.13 (4.33-13.9)† |
| Leukemia | 3 | 0.67 | 4.50 (0.93-13.2) |
| Complement deficiencies, n = 78 | |||
| All malignant neoplasms* | 1 | 2.13 | 0.47 (0.01-2.62) |
| NHL§ | 1 | 0.11 | 9.42 (0.24-52.5) |
| Leukemia | 0 | 0.04 | 0-92.3¶ |
| Combined T-cell and B-cell immunodeficiencies, n = 54 | |||
| All malignant neoplasms* | 0 | 0.32 | 0-11.7¶ |
| NHL | 0 | 0.03 | 0-117¶ |
| Leukemia | 0 | 0.01 | 0-395¶ |
| Other well-defined immunodeficiency syndromes, n = 66 | |||
| All malignant neoplasms* | 3 | 0.23 | 13.0 (2.69-38.1)† |
| NHL‖ | 2 | 0.03 | 62.1 (7.51-224)† |
| Leukemia | 0 | 0.01 | 0-376¶ |
| Congenital defects of phagocyte number, function or both, n = 39 | |||
| All malignant neoplasms* | 0 | 0.48 | 0-7.69¶ |
| NHL | 0 | 0.03 | 0-114¶ |
| Leukemia | 0 | 0.02 | 0-227¶ |
| Diseases of immune dysregulation, n = 14 | |||
| All malignant neoplasms* | 1 | 0.23 | 4.40 (0.11-24.5) |
| NHL | 0 | 0.01 | 0-281¶ |
| Leukemia | 1 | 0.004 | 238 (6.03-1326)† |
95% CI indicates 95% confidence interval; E, expected number of cases; NHL, non-Hodgkin lymphoma; O, observed number of cases; PID, primary immune deficiency; and SIR, standardized incidence ratio.
C00-C96, excluding C44.
Statistical significance.
Diffuse large B-cell lymphoma (n = 1), follicular lymphoma (n = 2), Burkitt lymphoma/leukemia (n = 1), small lymphocytic lymphoma (n = 1), extranodal marginal zone lymphoma type (n = 1), peripheral T-cell lymphoma (n = 1) unclassified, cutaneous T-cell lymphoma unclassified (n = 1), Waldenström macroglobulinemia (n = 1), and NHL unclassified (n = 4).
Diffuse large B-cell lymphoma (n = 1).
Follicular lymphoma (n = 1), NHL unclassified (n = 1).
One-tail, 97.5% CI instead of 95% CI because of zero observation.