Induction therapy for HLH. Based on the HLH-94 study, this approach should be considered standard of care for all patients not enrolled in clinical trials, based on published evidence of efficacy.71 Etoposide is dosed as 150 mg/m2 per dose. Alternatively, for patients weighing < 10 kg, consideration may be given to dosing etoposide as 5 mg/kg per dose. Dexamethasone (Dex.) is dosed as indicated and may be given orally or intravenously, although the latter is preferred at therapy initiation. Intrathecal methotrexate and hydrocortisone (IT MTX/HC) should be given to patients with evidence of CNS involvement, as early as LP may be safely performed (which may vary from the diagram) and dosed as follows: age < 1 year, 6/8 mg (MTX/HC); 1-2 years, 8/10 mg; 2-3 years, 10/12 mg; > 3 years, 12/15 mg. Weekly intrathecal therapy is generally continued until at least 1 week after resolution of CNS involvement (both clinical and CSF indices).
