Association of different parameters and outcome (n = 17)
| Parameter/category . | n . | Outcome . | ||
|---|---|---|---|---|
| Survivors (n = 7) . | Nonsurvivors (n = 10) . | P . | ||
| Phenotype | ||||
| CHARGE | 7 | 2 | 5 | NS |
| DGS | 10 | 5 | 5 | NS |
| Atypical DGS | 3 | 1 | 2 | NS |
| Gene defect | ||||
| 22q11.2 | 8 | 5 | 3 | NS |
| CHD7 | 4 | 3 | 1 | NS |
| Transplantation regimen | ||||
| GVHD prophylaxis | 11 | 4 | 7 | NS |
| Conditioning | 5 | 0 | 5 | .026 |
| Donor | ||||
| MSD | 7 | 5 | 2 | .034 |
| MRD | 1 | 0 | 1 | NS |
| Haploidentical | 1 | 0 | 1 | NS |
| MUD | 8 | 2 | 6 | NS |
| Graft | ||||
| Marrow | 9 | 3 | 6 | NS |
| Combined (PBL, then marrow) | 2 | 2 | 0 | NS |
| PBL | 5 | 2 | 3 | NS |
| Cord | 1 | 0 | 1 | NS |
| B cell–depleted PBL | 1 | 0 | 1 | NS |
| T cell–depleted marrow | 1 | 0 | 1 | NS |
| Age at transplantation | ||||
| Less than or equal to 3 months | 7 | 3 | 4 | NS |
| Median | 17 | 5 | 4.5 | NS |
| Immune reconstitution | ||||
| T cells/mm3 (median at the last follow-up) | 15 | 806 (5 patients) | 480 (10 patients) | NS |
| Complications | ||||
| GVHD (grade ≥ 2) | 9 | 3 | 6 | NS |
| Parameter/category . | n . | Outcome . | ||
|---|---|---|---|---|
| Survivors (n = 7) . | Nonsurvivors (n = 10) . | P . | ||
| Phenotype | ||||
| CHARGE | 7 | 2 | 5 | NS |
| DGS | 10 | 5 | 5 | NS |
| Atypical DGS | 3 | 1 | 2 | NS |
| Gene defect | ||||
| 22q11.2 | 8 | 5 | 3 | NS |
| CHD7 | 4 | 3 | 1 | NS |
| Transplantation regimen | ||||
| GVHD prophylaxis | 11 | 4 | 7 | NS |
| Conditioning | 5 | 0 | 5 | .026 |
| Donor | ||||
| MSD | 7 | 5 | 2 | .034 |
| MRD | 1 | 0 | 1 | NS |
| Haploidentical | 1 | 0 | 1 | NS |
| MUD | 8 | 2 | 6 | NS |
| Graft | ||||
| Marrow | 9 | 3 | 6 | NS |
| Combined (PBL, then marrow) | 2 | 2 | 0 | NS |
| PBL | 5 | 2 | 3 | NS |
| Cord | 1 | 0 | 1 | NS |
| B cell–depleted PBL | 1 | 0 | 1 | NS |
| T cell–depleted marrow | 1 | 0 | 1 | NS |
| Age at transplantation | ||||
| Less than or equal to 3 months | 7 | 3 | 4 | NS |
| Median | 17 | 5 | 4.5 | NS |
| Immune reconstitution | ||||
| T cells/mm3 (median at the last follow-up) | 15 | 806 (5 patients) | 480 (10 patients) | NS |
| Complications | ||||
| GVHD (grade ≥ 2) | 9 | 3 | 6 | NS |
DGS indicates DiGeorge syndrome; CHD, chromodomain helicase; MRD, matched related donor; MUD, matched unrelated donor; MSD, matched sibling donor; GVHD, graft-versus-host disease; PBL, peripheral blood lymphocyte; OBL, peripheral lymphocyte; and NS, statistically not significant.