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. 2019 Feb 26;39(Suppl 1):1–151. doi: 10.1007/s10875-019-00597-5

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]