Table 3.

Summary of the haemophagocytic lymphohistiocytosis (HLH)-2004 treatment protocol (the complete protocol is available on request at http://www.histiocytesociety.org).

Initial therapy (weeks 1–8)

Etoposide intravenous 150 mg/m2twice weekly for 2 weeks and then weekly

Dexamethasone intravenous daily 10 mg/ m2for 2 weeks, 5 mg/ m2for 2 weeks, 2·5 mg/ m2for 2 weeks, 1·25 mg/ m2for 2 weeks, tapering for 1 week

Cyclosporin A oral daily, aiming at trough levels of 200 µg/l

Intrathecal methotrexate (up to 4 doses) weeks 3–6 if there is central nervous system involvement

Continuation therapy (week 9 until haematopoietic stem cell transplant)

Etoposide 150 mg/m2every second week alternating with dexamethasone

Dexamethasone intravenous 10 mg/ m2× 3 days every second week

Cyclosporin A daily oral aiming at trough levels of 200 µg/l

Reactivation therapy

Restart from week 2 of initial therapy