Children's Cancer Research Institute, Vienna, Austria.
J Pediatr. 2012 Jul;161(1):129-33.e1-3. doi: 10.1016/j.jpeds.2011.12.035. Epub 2012 Jan 28.
To assess the effect of pulmonary involvement on the course and outcome of multisystem Langerhans cell histiocytosis (MS-LCH) in children.
We conducted a retrospective analysis of 420 consecutive patients with MS-LCH. In this analysis, the term "risk organs" is defined as involvement of the liver, spleen, and/or hematopoietic system. The effect of pulmonary involvement on survival was assessed with multivariate Cox regression with adjustment for risk organs involvement and age.
Pulmonary involvement in MS-LCH was present at diagnosis in 102 patients (24%). Of the 318 patients without pulmonary involvement at diagnosis, it developed in 28 within a median of 10 months (range, 1 month-5.5 years). The 5-year overall survival rate in patients without risk organ involvement at diagnosis was 96% in patients without pulmonary involvement and 94% in those with pulmonary involvement. In patients with risk organ involvement at diagnosis, the 5-year overall survival rate was 73% in patients without pulmonary involvement and 65% in patients with pulmonary involvement. In multivariate analysis, pulmonary involvement at diagnosis had no significant impact on survival rats (P = .109, hazard ratio = 1.5).
In multivariate analysis, pulmonary involvement was not an independent prognostic variable and should therefore be excluded from the definition of risk organ involvement in MS-LCH.
评估肺部受累对儿童多系统朗格汉斯细胞组织细胞增生症（MS-LCH）病程和结局的影响。
我们对 420 例连续 MS-LCH 患者进行了回顾性分析。在本分析中，“风险器官”一词定义为肝脏、脾脏和/或造血系统受累。采用多变量 Cox 回归分析，调整风险器官受累和年龄因素，评估肺部受累对生存的影响。
102 例（24%）患者在诊断时存在肺部受累。在 318 例无肺部受累的患者中，中位时间为 10 个月（范围为 1 个月至 5.5 年）后发生肺部受累。无风险器官受累的患者，无肺部受累和有肺部受累的患者 5 年总生存率分别为 96%和 94%。有风险器官受累的患者，无肺部受累和有肺部受累的患者 5 年总生存率分别为 73%和 65%。多变量分析显示，肺部受累对生存率无显著影响（P=.109，风险比=1.5）。
多变量分析显示，肺部受累不是独立的预后变量，因此不应将其纳入 MS-LCH 风险器官受累的定义中。
