Clinical, treatment, and outcome profilesa
| 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.