Brugières L, Valteau D, Patte C, Kalifa C, Hartmann O, Lemerle J
Ann Pediatr (Paris). 1989 Oct;36(8):493-6.
Thirty-two children with malignant histiocytosis were managed at the Department of Pediatrics of the Gustave-Roussy Institute from January 1975 through December 1987. Clinical presentation included enlarged lymph nodes in all cases, a decline in general health with fever in 74%, skin manifestations in 54%, and visceral involvement in 55%. Twenty-seven patients were treated with the COPAD protocol combining vincristine, cyclophosphamide, adriamycin and prednisone. Twenty-two patients achieved a complete remission, and one a partial remission; there was no remission in four patients. Eight patients had a recurrence and were given a combination of CCNU, vinblastine and bleomycin; seven achieved a second complete remission. Overall survival rate was 72.2% with a median follow up of five years. Survival rate free of disease was 54.5%. Poor prognostic factors included presence of constitutional symptoms upon diagnosis and age under ten years.
1975年1月至1987年12月期间，古斯塔夫 - 鲁西研究所儿科收治了32例恶性组织细胞增多症患儿。临床表现均有淋巴结肿大，74%的患儿全身健康状况下降并伴有发热，54%有皮肤表现，55%有内脏受累。27例患者采用了包含长春新碱、环磷酰胺、阿霉素和泼尼松的COPAD方案进行治疗。22例患者实现完全缓解，1例部分缓解；4例未缓解。8例复发患者接受了洛莫司汀、长春碱和博来霉素联合治疗；7例实现第二次完全缓解。中位随访五年，总生存率为72.2%。无病生存率为54.5%。预后不良因素包括诊断时存在全身症状和年龄小于10岁。
