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

✍ Scribed by Ceci, Adriana ;De Terlizzi, Marino ;Colella, Roberto ;Loiacono, Giuseppe ;Balducci, Donato ;Surico, Giammarco ;Castello, Manuel ;Testi, Anna Maria ;De Bernardi, Bruno ;Indolfi, Paolo ;Macchia, Pierantonio ;Madon, Enrico ;Mancini, Antonia ;Rosati, Domenico

• Publisher: John Wiley and Sons
• Year: 1993
• Tongue: English
• Weight: 568 KB
• Volume: 21
• Category: Article
• ISSN: 0098-1532
• DOI: 10.1002/mpo.2950210405

✦ Synopsis

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. © 1993 Wiley‐Liss, Inc.