Langerhans cell histiocytosis in childhood: results from the Italian Cooperative AIEOP-CNR-H.X '83 study

Med Pediatr Oncol. 1993;21(4):259-64. doi: 10.1002/mpo.2950210405.

Abstract

Ninety patients with biopsy-proven Langerhans cell histiocytosis (LCH) were enrolled from June, 1983, to December, 1988, in the multicenter AIEOP-CNR-H.X. '83 study. They were divided into two groups: poor prognosis (PP), comprising 11 children with organ dysfunction (OD), and good prognosis (GP), made up of 79 patients without OD. Eighty-four patients were evaluable for treatment results. Among GP patients, 16 with a single lesion received only local treatment, while 59 entered a clinical trial of immunotherapy and/or monochemotherapy with vinblastine (VBL). Nonresponders, sequentially received doxorubicin (ADM) and then etoposide (VP16). PP patients were treated with 4 week cycles of vincristine, ADM, cyclophosphamide, and prednisone for nine courses. The overall survival was 92.8% (100% for GP patients and 45.5% for PP patients) at 48 months. The complete response (CR) rates for immunotherapy, VBL, ADM, and VP16 were 10%, 62.9%, 42.8%, and 88.2%, respectively. Two of the 11 PP patients had a CR (18.2%), while six died and three are still alive with recurrent disease. The overall incidence of disease-related disabilities was 47.7%, while that of diabetes insipidus was 20%. Monochemotherapy is probably adequate in GP patients, while more effective treatments are needed for PP patients.

Publication types

  • Clinical Trial
  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Antineoplastic Combined Chemotherapy Protocols / adverse effects
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Cyclophosphamide / administration & dosage
  • Doxorubicin / adverse effects
  • Doxorubicin / therapeutic use
  • Etoposide / adverse effects
  • Etoposide / therapeutic use
  • Female
  • Histiocytosis, Langerhans-Cell / therapy*
  • Humans
  • Immunotherapy
  • Infant
  • Infant, Newborn
  • Italy
  • Male
  • Prednisone / administration & dosage
  • Prognosis
  • Prospective Studies
  • Treatment Outcome
  • Vinblastine / adverse effects
  • Vinblastine / therapeutic use
  • Vincristine / administration & dosage

Substances

  • Vincristine
  • Vinblastine
  • Etoposide
  • Doxorubicin
  • Cyclophosphamide
  • Prednisone