Treatment of recurrent, refractory, or progressive high-risk disease: multisystem LCH

Data from the DAL group studies showed that patients with high-risk multisystem LCH who had progressive disease by week 6 of standard induction treatment or who did not achieve at least a partial response by week 12 had only a 10% chance of survival.[27] These results were consistent with those of the less-intensive HISTSOC-LCH-II trial in which patients treated with vinblastine/prednisone who did not respond well by week 6 had a 27% chance of survival, compared with 52% for good responders.[28][Level of evidence A1] To improve on these results, patients with poorly responsive disease need to move to salvage strategies by week 6 for progressive disease and no later than week 12 for those without at least a good response.

Treatment options for patients with recurrent, refractory, or progressive high-risk multisystem LCH include the following: