17例成人朗格汉斯细胞组织细胞增生症患者放射治疗的疗效分析

Efficacy of radiotherapy for adult patients with Langerhans cell histiocytosis

目的分析成人朗格汉斯细胞组织细胞增生症 （ Langenhans cell histiocytosis, LCH ）的放疗效果。方法回顾性分析2000年1月至2012年9月病理确诊的17例成人LCH患者的临床特征和放疗效果。结果17例患者中男11例，女6例，中位年龄31（18～56）岁，所有患者均为单系统受累起病。起病至放疗的中位时间为8．3（0～108）个月。17例患者中12例近期治疗有效。然而，16例患者放疗后疾病进展，中位疾病无进展生存时间为14（0～131）个月，仅1例保持疾病稳定。进展的16例患者中，1例为原位病变进展，15例出现新发病灶，最终多系统受累者13例。结论成人LCH患者行放疗后多数短期可以缓解症状，减少器官功能受损，但远期复发和放疗野外进展率较高。

Objective To analyze efficacy of radiotherapy for adult patients with langerhans cell histiocytosis （LCH）. Methods Clinical features and efficacy of radiotherapy for biopsy-proven adult patient with LCH from January 2000 to October 2012 in our hospital were retrospectively analyzed. Results Seven- teen （11 male and 6 female） adult LCH patients with a mean age of 31 （18 -56） years old were treated by irradiation, all patients presented as single-system disease. The mean duration from diagnosis to irradiation was 8.3 （0 - 108） months. Although 12 of 17 patients （70.6%） had short-term response to radiotherapy, all patients but one （ 94. 1% ） progressed during long-term follow-up, the mean progression-free survival （PFS） was 14 （0 - 131 ） months. Of the progressed patients, one relapsed in situ, the remaining 15 patients progressed outside the irradiated region. Thirteen patients （76.5%） eventually progressed to muhisystem disease. Conclusions Though radiotherapy for LCH in adults produced a high short-term response up to 70.6%, most of patients eventually progressed in situ or outside the irradiation region during long-term follow-up.