Ceci A
Pediatr Med Chir. 1984 Mar-Apr;6(2):185-91.
A number of factors should influence the choice of therapy in H.X. The three most important are: etiology, natural history and prognostic factors. At this time, we can identify 2 groups of patients: - the first is characterized by age greater than 2 years, bone involvement, absence of organ dysfunction, long survival, low mortality and high frequency of late effects (Good Risk Group); - the second group by age less than 2 years, soft tissue (+/- bone) involvement, presence of organ dysfunction and high mortality (Poor Risk Group). Chemotherapy appears to improve survival and Complete Remission rate in the Poor Risk Group but not in the Good Risk Group. New advances, i.e. immunotherapy, are in progress to reduce mortality in Poor Risk patients, late effects in Good Risk patients and related therapy sequelae.
多种因素应会影响HX患者的治疗选择。其中最重要的三个因素是：病因、自然病史和预后因素。目前，我们可以识别出两组患者：第一组的特征为年龄大于2岁、有骨骼受累、无器官功能障碍、生存期长、死亡率低且迟发效应发生率高（低危组）；第二组的特征为年龄小于2岁、有软组织（±骨骼）受累、存在器官功能障碍且死亡率高（高危组）。化疗似乎可提高高危组患者的生存率和完全缓解率，但对低危组患者无效。免疫疗法等新进展正在进行中，目的是降低高危患者的死亡率、低危患者的迟发效应以及相关治疗后遗症。
