LANGERHANS CELL HISTIOCYTOSIS IN ADULTS - A CLINICAL AND THERAPEUTIC ANALYSIS OF 11 PATIENTS FROM A SINGLE INSTITUTION

BACKGROUND. Langerhans' cell histiocytosis (LCH) is a rare disorder of uncertain etiology, characterized by a wide clinical spectrum and var ied behavior. METHODS. This retrospective study analyzed 11 adult pati ents with a diagnosis of LCH observed at the study institution between April 1988 and March 1993. RESULTS. Based on the sites and extent of disease at diagnosis, patients were divided into four categories. Grou p A was comprised of four patients with unifocal bone disease who had surgical curettage. At last follow-up only 1 patient was in continuous complete response (CCR) at 29+ months. The other 3 patients recurred at 3, 12, and 30 months, respectively, after surgery and at last follo w-up were found to be in CR at 16+, 48+, and 124+ months, respectively , after therapy with vinblastine (VBL) and high dose methylprednisolon e (HDMP). Group B was comprised of three patients with multifocal bone disease. Two of these patients received VBL + HDMP; at last follow-up , 1 patient tvas in CCR 8 months after completion of therapy, and the other developed progressive disease 11 months later. The third patient was treated with interferon (IFN) and at last follow-up was in CCR at 35+ months. Group C was comprised of 2 patients with bone and viscera l disease who were treated with etoposide (VP-16) + HDMP; at last foll ow-up, 1 patient was in CCR at 42+ months and the other patient, who h ad isolated vulvar recurrence 16 months later, was in CR with treatmen t with local IFN. Group D was comprised of two patients with lung and lymph node involvement, one of whom was treated with VP-16 + HDMP and the other with cyclophosphamide, doxorubicin, vincristine, and prednis one; at last follow-up, both were in CCR at 30+ and 71+ months, respec tively. CONCLUSIONS. VBL + HDMP showed efficacy in patients with bone disease, in particular those treated for recurrent LCH after surgery. Therapy with VP-16 and HDMP was successfully employed in patients with visceral disease. IFN was effective both for localized disease and in patients with multiple bone lesions. (C) 1997 American Cancer Society .