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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)