Table 1.
Patient and transplantation characteristics
| All patients, n (%)∗ |
HLH, n (%)∗ |
Other IEIs, n (%)∗ |
|
|---|---|---|---|
| Total patients | 33 (100%) | 25 (100%) | 8 (100%) |
| Diagnosis | |||
| HLH | 25 (76%) | 25 (100%) | 0 |
| CAEBV | 3 (9%) | 0 | 3 (38%) |
| CGD | 2 (6%) | 0 | 2 (25%) |
| HIGM | 2 (6%) | 0 | 2 (25%) |
| IPEX | 1 (3%) | 0 | 1 (12%) |
| Sex | |||
| Male | 22 (67%) | 17 (68%) | 5 (62%) |
| Female | 11 (33%) | 8 (32%) | 3 (38%) |
| Race | |||
| White | 27 (82%) | 20 (80%) | 7 (88%) |
| Non-White or not reported | 6 (18%) | 5 (20%) | 1 (12%) |
| Age at transplantation, y | |||
| <1 | 10 (30%) | 10 (40%) | 0 |
| 1-16 | 18 (55%) | 13 (52%) | 5 (63%) |
| 17-22 | 5 (15%) | 2 (8%) | 3 (37%) |
| Stem cell source | |||
| Marrow | 33 (100%) | 25 (100%) | 8 (100%) |
| Donor relation | |||
| Sibling | 5 (15%) | 5 (20%) | 0 |
| Other related | 1 (3%) | 0 | 1 (12%) |
| Unrelated | 27 (82%) | 20 (80%) | 7 (88%) |
| Patient/Donor HLA match | |||
| 8/8 or 6/6 | 21 (64%) | 16 (64%) | 5 (63%) |
| 7/8 | 12 (36%) | 9 (36%) | 3 (37%) |
CAEBV, chronic active Epstein-Barr virus disease; CGD, chronic granulomatous disease; HIGM, hyperimmunoglobulin M syndrome; IPEX, immune dysregulation, polyendocrinopathy, and enteropathy, X-linked syndrome.
∗
Column percentage given.