Table 4.
Clinical characteristics, treatment and outcome of patients with sHLH (n = 35)
| sHLH with genetic variants n = 3 |
sHLH not found to harbor any mutation and not genetically tested n = 32 (14 + 18) |
|
|---|---|---|
| Age (year) | ||
| Median | 5.6 | 5.4 |
| Average | 5.8 | 6.7 |
| SD | 4.4 | 4.9 |
| Range | 1.5–10 | 0.5 – 18 |
| Sex (M/F) | 2/1 | 17/15 |
| Trigger | EBV – 2 | EBV – 14 |
| HHV6 – 1 | HHV6 – 4 | |
| CMV – 1 | ||
| Parvovirus – 1 | ||
| RSV – 1 | ||
| Mycoplasma pn. – 1 | ||
| Chlamydia – 1 | ||
| Streptococcus pn. – 1 | ||
| Unknown – 8 | ||
| Functional studies | ||
| Low NK activity | 1/2 | 18/24 |
| Abnormal degranulation | nd | 7/20 |
| Decreased perforin | nd | 2/21 |
| sIL-2R | nd | Nd |
| Treatment | ||
| HLH-2004 | 2 | 23 |
| Dexamethazone + CSA + IVIg | 5 | |
| Dexamethazone | 1 | 3 |
| IVIG | 1 | |
| SCT | 2 | 8 |
| Outcome | ||
| Alive without SCT | 1 | 20 |
| Alive after SCT | 2 | 7 |
| Death before SCT | 0 | 4 |
| Death after SCT | 0 | 1 |
RSV respiratory syncytial virus, CMV cytomegalovirus, EBV Epstein-Barr virus, nd not determined