Fig. 1.
Overview of the treatment protocol HLH-94.
Day of death for the 23 patients who died the first year of therapy, except those who underwent BMT, is marked with an exclamation point (!). (BMT = Go to BMT during continuation therapy as soon as an acceptable donor is available, preferably when the disease is nonactive. The patients without either familial or persistent disease were recommended to cease therapy after the initial therapy and restart in case of reactivation. Dexa = dexamethasone daily [pulses are 10 mg/m2 for 3 days]; VP-16 = etoposide 150 mg/m2 intravenously; CSA = cyclosporin A; I.T. therapy = intrathecal methotrexate [if progressive neurological symptoms or if an abnormal CSF has not improved].)