Table 1.
Patient cohort
| Total | Lowest DC ≤30% | DC always >30% | P value | |
|---|---|---|---|---|
| n | 103 | 56 | 47 | |
| Male/female, % | 65/35 | 70/30 | 60/40 | NS |
| Age at diagnosis, months, median (range) | 5 (0-288) | 5 (1-288) | 4 (0-144) | NS |
| Patient with HLH onset in infancy, % | 67 | 61 | 74 | NS |
| Age at HSCT, months, median (range) | 12 (2-236) | 12 (3-326) | 10 (2-150) | NS |
| Distribution of defects, % of group | ||||
| FHL2 | 15 | 14 | 17 | NS |
| FHL3 | 27 | 31 | 23 | NS |
| FHL4 | 3 | 2 | 4 | NS |
| FHL5 | 16 | 23 | 9 | NS |
| GS2 | 9 | 5 | 13 | NS |
| CHS | 3 | 5 | 0 | NS |
| XLP1 | 5 | 2 | 9 | NS |
| XIAP deficiency (XLP2) | 4 | 4 | 4 | NS |
| Others/not known, diagnosed clinically | 18 | 14 | 21 | NS |
| Donor, % of group | ||||
| Matched related donor | 24 | 18 | 32 | NS |
| Matched unrelated donor | 45 | 44 | 45 | NS |
| Mismatched donor | 29 | 34 | 23 | NS |
| Haploidentical donor | 2 | 4 | 0 | NS |
| Conditioning regimen, % of group | ||||
| MAC | 25 | 27 | 23 | NS |
| RIC | 74 | 71 | 77 | NS |
| Other | 1 | 2 | 0 | NS |
Patient characteristics are shown for the entire cohort, for patients in whom the lowest DC was ≤30%, and for patients in whom DC was always >30%. There were no significant differences between both groups.
NS, not significant.