title: Etoposide as the basic and interferon-alpha as the maintenance therapy for Langerhans cell histiocytosis: a RTC.
abstract: The treatment of patients who suffer from a disseminated form of Langerhans cell histiocytosis (LCH) is still controversial. So far, few larger randomized studies have been performed. The authors present 3 patients with a disseminated form of LCH--4 months, 9 months, and 2 years old, respectively. The lesional Langerhans cells in each patient showed positive immunohistochemical reaction to S-100 protein and the presence of Birbeck granules was confirmed by electron microscopy. All the patients were treated with etoposide (VP-16), 200 mg/m2 for 3 consecutive days, with 15 cycles at intervals of 3 weeks between each cycle, followed by maintenance therapy with IFN-alpha. All 3 patients reached complete stabile remission. The patients were young, at high risk, with multiple-organ involvement of LCH, and two of them had obvious signs of organ dysfunction at presentation, suggesting a poor prognosis. All remain disease-free several years after therapy. The results suggest that INF-alpha may prevent recurrences in high-risk patients.
authors.affiliation: Department of Pediatrics, Pediatric Hemato-Oncology, Clinical Hospital Split, Split, Croatia. srdjana.culic@st.tel.hr
authors.forename: S
authors.initials: S
authors.lastname: Culić
authors.forename: A
authors.initials: A
authors.lastname: Jakobson
authors.forename: V
authors.initials: V
authors.lastname: Culić
authors.forename: I
authors.initials: I
authors.lastname: Kuzmić
authors.forename: M
authors.initials: M
authors.lastname: Sćukanec-Spoljar
authors.forename: D
authors.initials: D
authors.lastname: Primorac
mesh_terms: D000914:Antibodies, Viral / Q000097:blood; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D002675:Child, Preschool; D003587:Cytomegalovirus / Q000276:immunology; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D004854:Herpesvirus 4, Human / Q000276:immunology; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology / Q000821:virology; D006801:Humans; D007223:Infant; D016898:Interferon-alpha / Q000008:administration & dosage; D012074:Remission Induction; D009418:S100 Proteins / Q000032:analysis
chemical_list: D000914:Antibodies, Viral; D016898:Interferon-alpha; D009418:S100 Proteins; D005047:Etoposide