Site-specific and overall cancer risk for the most common individual PID conditions
| PID condition/cancer site . | O . | E . | SIR (95% CI) . |
|---|---|---|---|
| Common variable immune deficiency, n = 416 | |||
| All malignant neoplasms* | 38 | 19.6 | 1.94 (1.37-2.67)† |
| NHL‡ | 11 | 0.91 | 12.1 (6.03-21.6)† |
| Leukemia | 1 | 0.40 | 2.49 (0.06-13.9) |
| Stomach | 2 | 0.28 | 7.23 (0.88-26.1) |
| Thymus | 2 | 0.01 | 146 (17.7-528)† |
| Breast (female only) | 9 | 4.03 | 2.24 (1.02-4.24)† |
| IgG subclass deficiency, n = 244 | |||
| All malignant neoplasms* | 11 | 8.83 | 1.32 (0.66-2.36) |
| Leukemia | 2 | 0.15 | 13.2 (1.60-47.6)† |
| Breast (female only) | 3 | 2.10 | 1.43 (0.29-4.17) |
| Selective IgA deficiencies, n = 90 | |||
| All malignant neoplasms* | 0 | 3.58 | 0-1.03¶ |
| X-linked agammaglobulinemia, n = 62 | |||
| All malignant neoplasms* | 1 | 0.32 | 3.09 (0.08-17.2) |
| Stomach | 1 | 0.004 | 236 (5.97-1315)† |
| Ataxia-telangiectasia, n = 10 | |||
| All malignant neoplasms* | 2 | 0.05 | 41.2 (4.99-149)† |
| NHL§ | 1 | 0.006 | 165 (4.19-922)† |
| PID condition/cancer site . | O . | E . | SIR (95% CI) . |
|---|---|---|---|
| Common variable immune deficiency, n = 416 | |||
| All malignant neoplasms* | 38 | 19.6 | 1.94 (1.37-2.67)† |
| NHL‡ | 11 | 0.91 | 12.1 (6.03-21.6)† |
| Leukemia | 1 | 0.40 | 2.49 (0.06-13.9) |
| Stomach | 2 | 0.28 | 7.23 (0.88-26.1) |
| Thymus | 2 | 0.01 | 146 (17.7-528)† |
| Breast (female only) | 9 | 4.03 | 2.24 (1.02-4.24)† |
| IgG subclass deficiency, n = 244 | |||
| All malignant neoplasms* | 11 | 8.83 | 1.32 (0.66-2.36) |
| Leukemia | 2 | 0.15 | 13.2 (1.60-47.6)† |
| Breast (female only) | 3 | 2.10 | 1.43 (0.29-4.17) |
| Selective IgA deficiencies, n = 90 | |||
| All malignant neoplasms* | 0 | 3.58 | 0-1.03¶ |
| X-linked agammaglobulinemia, n = 62 | |||
| All malignant neoplasms* | 1 | 0.32 | 3.09 (0.08-17.2) |
| Stomach | 1 | 0.004 | 236 (5.97-1315)† |
| Ataxia-telangiectasia, n = 10 | |||
| All malignant neoplasms* | 2 | 0.05 | 41.2 (4.99-149)† |
| NHL§ | 1 | 0.006 | 165 (4.19-922)† |
95% CI indicates 95% confidence interval; E, expected number of cases; IgA, immunoglobulin A; IgG, immunoglobulin G; 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, Waldenström macroglobulinemia (n = 1), and NHL unclassified (n = 3).
NHL unclassified (n = 1).
One-tail, 97.5% CI instead of 95% CI because of zero observation.