Optimal treatment for Langerhans cell histiocytosis (LCH) has not yet been established. High-risk patients with systemic LCH may have a fatal course of the disease despite intensive treatment. New approaches using cyclosporin A (CSA) showed promising results. Here, we report on a 4-year-old boy who presented with systemic LCH of skin, liver, bone, bone marrow, and soft tissue infiltrates. The patient was refractory to conventional therapy including VP16, prednisone, 6-mer-captopurine, methotrexate, and vinblastine. Therefore the patient was treated with CSA as continuous therapy (serum levels were kept between 300 and 400 ng/mL) as well as intensification with VP16 and prednisone every 4 weeks. As early as 4 months after starting this treatment, clinical symptoms completely disappeared except for a slightly enlarged liver. During the next 12 months all clinical symptoms except a limited skin involvement vanished although treatment with VP16 and prednisone was stopped and CSA serum levels were kept between 100 and 150 mg/mL. In conclusion, intensive therapy using high-dose CSA combined with VP16 and prednisone might be a therapeutic option for patients with otherwise refractory LCH.
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