Table 1.

Summary of HLH-2004 treatment protocol.3 Supportive care with prophylactic co-trimoxazole, anti-virals (eg aciclovir), intravenous immunoglobulin (0.5 g/kg 4-weekly) and anti-fungals are also administered

Week of treatmentTreatment
Initial treatment
1–2Dexamethasone 10 mg/m2
Etoposide 150 mg/m2 on day 1, 4, 8 and 11
Ciclosporin 3 mg/kg twice daily aiming for a trough level 200 mcg/L
3–4Dexamethasone 5 mg/m2
Etoposide 150 mg/m2 weekly
Ciclosporin 3 mg/kg twice daily aiming for a trough level 200 mcg/L
Intrathecal methotrexate 12 mg with hydrocortisone 50 mg at week 3 (repeat weekly on week 4–6 if evidence of central nervouse system involvement)
5–6Dexamethasone 2.5 mg/m2
Etoposide 150 mg/m2 weekly
Ciclosporin 3 mg/kg twice daily aiming for a trough level 200 mcg/L
7–8Dexamethasone 1.25 mg/m2
Etoposide 150 mg/m2 weekly
Ciclosporin 3 mg/kg twice daily aiming for a trough level 200 mcg/L
Continuation therapy
9 onwardsDexamethasone 10 mg/m2 for 3 days every 2 weeks
Etoposide 150 mg/m2 every 2 weeks
Ciclosporin 3 mg/kg twice daily aiming for a trough level 200 mcg/L