TREATMENT STRATEGY FOR DISSEMINATED LANGERHANS CELL HISTIOCYTOSIS

被引:228
作者
GADNER, H [1 ]
HEITGER, A [1 ]
GROIS, N [1 ]
GATTERERMENZ, I [1 ]
LADISCH, S [1 ]
机构
[1] CHILDRENS NATL MED CTR,CTR CANC & TRANSPLANTAT BIOL,WASHINGTON,DC
来源
MEDICAL AND PEDIATRIC ONCOLOGY | 1994年 / 23卷 / 02期
关键词
CHEMOTHERAPY; DIABETES-INSIPIDUS; MORTALITY; VP16; PERMANENT CONSEQUENCES;
D O I
10.1002/mpo.2950230203
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
Treatment of Langerhans cell histiocytosis (LCH) remains problematic. To test the hypothesis that rapid initiation and long-term continuation of chemotherapy can improve survival and reduce recurrence and late consequences of disseminated LCH, we have completed a prospective clinical trial (DAL HX-83). One hundred six newly diagnosed patients were stratified into three risk groups (A: multifocal bone disease [n = 28]; B: soft tissue involvement without organ dysfunction [n = 57]; C: organ dysfunction [n = 21]). All patients received an identical initial 6-week treatment (etoposide [VP-16], prednisone, and vinblastine), and continuation treatment for 1 year, slightly adapted according to stratification at diagnosis. It included oral 6-mercaptopurine and eight pulses of vinblastine and prednisone for all patients, plus VP-16 in group B and VP-16 and methotrexate in group C. Eighty-nine percent and 91% of patients in groups A and B and 67% of the most severely affected group C, achieved complete resolution of disease. The speed of resolution was rapid (median 4 months) and independent of disease severity. The frequency of recurrence after initial resolution was low (12%, 23%, and 42% in groups A, B, and C); overall fully 77% of patients have remained free of recurrence. Permanent consequences developed after diagnosis in 20% of the patients. Diabetes insipidus after initiation of treatment occurred in only 10% of patients. Mortality (9%) was limited to patients of groups B (two patients) and C (eight patients). Finally, among the 106 patients treated by DAL HX-83 none have developed a malignancy (median follow-up 6 years, 9 months). The shorter duration of active disease, low rate of recurrence and permanent consequences, and improved survival among patients with poor prognosis support the strategy of rapid initiation of a predefined prolonged treatment upon the diagnosis of disseminated LCH. (C) 1994 Wiley-Liss, Inc.
引用
收藏
页码:72 / 80
页数:9
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