Table 1.
Patient characteristics
| UPN | Donor | Age at diagnosis | Genetics | Clinical complications prior to HSCT | Lung function prior to HSCT |
|---|---|---|---|---|---|
| 190 | MSD | 1.5 | XL (gp91phox) | Lung aspergillosis, suppurative lymphadenitis, liver and lung abscesses | Normal |
| 504 | MSD | 2 | XL (gp91phox) | Suppurative lymphadenitis, no pulmonary complications | Normal |
| 521 | MSD | 13 | XL (gp91phox) | Lung aspergillosis, skin abscesses, osteomyelitis | Restricted |
| 461 | MUD | 2 | XL (gp91phox) McLeod | Lung aspergillosis, granulomas (lungs and CNS) | Restricted |
| 483 | MUD | 3 | XL (gp91phox) | Lung aspergillosis, perianal abscess, granulomas (lungs and GIT), segmental lung resection | Restricted |
| 494 | MUD | 2 | XL (gp91phox) | Lung aspergillosis, liver abscess, granulomas (lungs) | Restricted |
| 508 | MUD | 0.3 | XL (gp91phox) | Skin, liver and spleen abscesses, granulomas (lungs) | Normal |
| 513 | MUD | 1 | XL (gp91phox) | Lung aspergillosis, suppurative lymphadenitis, osteomyelitis, glomerulonephritis | Restricted |
| 544 | MUD | 2 | XL (gp91phox) McLeod | Lung aspergillosis, BCGitis, segmental lung resection | Normal |
| 603 | MUD | 3 | XL (gp91phox) | Lung aspergillosis, suppurative lymphadenitis, perianal abscess | Normal |
| 614 | MUD | 3 | AR (p47phox) | Lung aspergillosis, skin abscess, granulomas (lungs, skin) | Restricted |
| 621 | MUD | 5.6 | XL (gp91phox) | Lung nocardiosis | Severely restricted |
UPN unique patient number, MSD matched sibling donor, MUD matched unrelated donor, HSCT hematopoietic stem cell transplantation, GIT gastrointestinal tract, CNS central nervous system, XL X-linked inheritance, McLeod phenotype: deletion of the Xk-gene next to the gp91phox-gene leads to reduced expression of Kell-antigens