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Table 1: Clinical, treatment, and outcome profiles^{^a}
Profiles 
Age
 Age at onset (median) (IQR) (mo) 36 (5.5–80.8)
 Age at CNS presentation (median) (IQR) (mo) 49.2 (11–96)
Male/female (ratio) 34:23 (1.4:1)
General symptoms
 Fever 45/56 (80)
 Hepato-/splenomegaly 46/56 (82)
 Abdominal distension 14/56 (25)
CNS symptoms 45/57, 79%
 Seizures 28 (62)
 Decreased sensorium 22 (49)
 Meningismus 13 (29)
 Gait ataxia 12 (27)
 Hypotonia 11 (24)
Minimal symptoms (mild irritability) or clinically silent patients (no symptoms with CSF abnormalities) 12 (21)
CSF findings
 CSF analyzed at presentation 50/57 (87)
 Abnormal CSF 42/50 (84)
 CSF pleocytosis (>10 leucocytes/μL) 25/50 (50)
 CSF proteinosis (>45 mg/dL) 32/50 (64)
Treatment
 HLH 1994/2004 35/57 (61)
 IT methotrexate 17 (30)
 HSCT 25 (45)
Outcome profiles
 Death 19 (32)
  Death before 8 weeks 7
  Death after 8 weeks 12
Alive at last follow-up 36 (63)
Lost to follow-up 2
Note:—IQR indicates interquartile range; IT, intrathecal.
↵a Total (n = 57). Data collected are both continuous and categorical, and the analysis method used has been referred to under statistical analysis heading.