Table 4.
Comparison of DC Patients with Pulmonary Disease with and without HSCT*
| Parameter | HSCT | No HSCT | **P value |
|---|---|---|---|
| Number with pulmonary disease/total number*** | 12/70 | 37/488 | 0.03 |
| Odds Ratio for pulmonary disease vs. no pulmonary disease, in patients with HSCT | 2.2 (1-4.5) | - | 0.03 |
| Age (years) at pulmonary disease in those who developed pulmonary disease, median (range) | 14 (4-36) | 37 (7-68) | <0.001 |
| Median survival age (years) free of pulmonary disease | 34 | 61 | <0.001 |
| Interval (years) from HSCT to pulmonary disease, median (range) | 4.5 (0-20) | - | - |
| Number who died | 9/12 | 24/37 | 0.8 |
| Interval (years) from pulmonary disease to death in those who developed pulmonary disease, median (range) | 0.3 (0-2) | 0 (0-8) | 0.5 |
| Median survival interval (years) after pulmonary disease, | 1 | 2 | 0.3 |
* Data obtained from the cases in Tables 2 and 3.
**P values compare the patients who received HSCT with those who did not receive HSCT.
*** The denominator is the total number of patients with DC who did or did not receive HSCT.
Abbreviations: DC, dyskeratosis congenita; HSCT, hematopoietic stem cell transplantation.