All Patients (N=34) |
Patients Failing Conventional Therapy (n=27) |
|
Age at HLH diagnosis, years (median [range]) | 0.85 [0.03, 13.0] | 1.00 [0.2-13.0] |
Sex (n [%]) | ||
Male | 16 [47.1] | 11 [40.7] |
Female | 18 [52.9] | 16 [59.3] |
HLH Genetic Confirmation (n [%]) | 27 [79.4] | 22 [81.5] |
FHL1 | 2 [5.9] | 0 |
FHL2 | 7 [20.6] | 5 [18.5] |
FHL3 | 8 [23.5] | 7 [25.9] |
FHL4 | 1 [2.9] | 1 [3.7] |
FHL5 | 2 [5.9] | 2 [7.4] |
Griscelli Syndrome type 2 | 5 [14.7] | 5 [18.5] |
X-linked Lymphoproliferative Disorder 1 | 1 [2.9] | 1 [3.7] |
X-linked Lymphoproliferative Disorder 2 | 1 [2.9] | 1 [3.7] |