title: Treatment of relapsed Langerhans cell histiocytosis by cyclosporin A combined with etoposide and prednisone.
abstract: 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.
authors.affiliation: Department of Pediatric Hematology and Oncology, Heinrich-Heine University Medical Center, Düsseldorf, Germany.
authors.forename: D
authors.initials: D
authors.lastname: Körholz
authors.forename: G
authors.initials: G
authors.lastname: Janssen
authors.forename: U
authors.initials: U
authors.lastname: Göbel
mesh_terms: D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use*; D000972:Antineoplastic Agents, Phytogenic / Q000627:therapeutic use*; D016572:Cyclosporine / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005047:Etoposide / Q000627:therapeutic use*; D006646:Histiocytosis, Langerhans-Cell / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D008297:Male; D011241:Prednisone / Q000627:therapeutic use*; D012008:Recurrence
chemical_list: D018931:Antineoplastic Agents, Hormonal; D000972:Antineoplastic Agents, Phytogenic; D007166:Immunosuppressive Agents; D005047:Etoposide; D016572:Cyclosporine; D011241:Prednisone