TABLE 1.
Clinical and laboratory characteristics of adult and pediatric HLH patients
| Adults treated with etoposide‐based therapy | Paediatric patients | |
|---|---|---|
| Number | 23 | 10 |
| Age (median, range) | 54 (21‐80) | 10 (2 months‐17 years) |
| Genetic testing (number of patients tested; number positive) | Three patients tested (Cincinnati panel) with no identifiable mutations |
Three patients tested One patient homozygous for MUNC13‐4 gene mutation (c.753+3G > A) |
| Ferritin (µg/L); median, range | 20 071 (3773‐321000) | 937 (80‐45700) |
| sIL‐2r (U/mL); median, range | 14 524 (2650‐39439) | 4 479 (3379‐6039) |
| LDH (U/L); median, range | 1 343 (264‐19200) | 3 175 (127‐17711) |
| Infectious trigger? (including EBV) | 11/23(48%) | 4/10 |
| EBV Positive | 7/23 | 2/10 |
| Etoposide‐based treatment | 23/23 (From 45 total adult patients over the same time period) | 5/10 HLH 2004 Protocol |
| Survival at 30 days | 18/23 (78%) | 10/10 |
| Survival at 90 days | 12/23 (52%) | 10/10 |
| Survival at one year | 7/21 (29%) | 7/10 |
| Hematopoietic stem cell transplant | 2/23 | 3/10 |
| Median follow up (years) | 3.7 (1‐4) | 4.8 (1‐10) |