Table 1.
HLH patient characteristics
| Patient characteristics | HLH (n = 122) |
|---|---|
| Age at diagnosis, median (range), y | 6.07 (0.04-18.50) |
| Sex, n (%) | |
| Male | 62 (50.82) |
| Female | 60 (49.18) |
| Race/ethnicity, n (%) | |
| Non-Hispanic white | 24 (19.67) |
| Hispanic | 52 (42.62) |
| Non-Hispanic black | 19 (15.57) |
| Non-Hispanic Asian | 6 (4.92) |
| Non-Hispanic other | 2 (1.64) |
| Other | 13 (9.84) |
| Unknown | 7 (5.74) |
| HLH genetic profile, n (%) | |
| fHLH | 19 (15.57) |
| PIDD | 11 (9.02) |
| DIAP | 8 (6.56) |
| PIDD/DIAP | 3 (2.46) |
| Other candidate defects | 5 (4.10) |
| No genetic explanation | 76 (62.30) |
| No biallelic fHLH gene variants | 63 (51.64) |
| One fHLH gene variant | 6 (4.92) |
| Possible digenic fHLH variants | 7 (5.74) |
| HLH-related trigger, n (%) | |
| Infection | 44 (36.07) |
| Autoimmune | 32 (26.23) |
| Malignancy | 14 (11.48) |
| No associated trigger | 32 (26.23) |
| Therapeutic strategy, n (%) | |
| Observation only | 9 (7.38) |
| Biologics/steroids | 18 (14.75) |
| Immunochemotherapy | 54 (44.26) |
| HSCT | 38 (31.15) |
| Unknown | 3 (2.46) |
| Alive at end of follow-up, n (%) | |
| Yes | 76 (62.30) |
| No | 41 (33.61) |
| Unknown | 5 (4.10) |