Table 2: Treatment of LCH high-risk multisystem disease

Spleen, liver, bone marrow or lung (may or may not include skin, bone, lymph node or pituitary gland).

Cytosine arabinoside, vincristine, and prednisolone followed by 6 months of maintenance therapy with cytarabine, vincristine, prednisolone and low-dose intravenous methotrexate. Patients had a poor response to the initial regimen, they were switched to a salvage regimen of intensive combination doxorubicin, cyclophosphamide, methotrexate, vincristine and prednisolone.