Table 3.
Primary disease |
New malignancy | Obs * |
Exp* | ObsExp (95% CI) | p-value | AER* |
---|---|---|---|---|---|---|
CGD | Melanoma of skin | 1 | 0.0027 | 370.33 (9.37, 2063) | 0.005 | 1.40 |
WAS | Kidney | 1 | 0.0656 | 15.23 (0.39, 84.85) | 0.127 | 1.31 |
HLH | Brain stem glioma | 1 | 0.0212 | 46.98 (1.19, 262) | 0.042 | 1.37 |
HLH | Hodgkin lymphoma | 1 | 0.0727 | 13.75 (0.35, 76.63) | 0.140 | 1.30 |
WAS | AML | 2 | 0.0533 | 37.55 (4.55, 135.66) | 0.003 | 2.72 |
CHS | MDS | 1 | 0.0516 | 19.38 (0.49, 108) | 0.101 | 1.33 |
LCH | Mucoepidermoid cancer | 1 | 0.2043 | 4.89 (0.12, 27.27) | 0.369 | 1.11 |
Abbreviations: 95% CI = 95% confidence interval; CGD=chronic granulomatous diseases; WAS=Wiskott Aldrich syndrome; HLH=hemophagocytic lymphohistiocytosis; SCID=T (−) B(+) severe combined immunodeficiency; CHS=Chediak Higashi syndrome; LCH=Langerhan cell histiocytosis;
AML=acute myeloid leukemia; MDS=myelodysplastic syndrome
Obs denotes observed cases and
Exp denotes expected cases
AER denotes absolute excess risk; number of observed cases minus number of expected cases per 10,000 person-years at risk