Lahey M E
Am J Pediatr Hematol Oncol. 1981 Spring;3(1):57-60.
It is now clear that the prognosis in children with histiocytosis X has improved considerable over the past few years. To be sure, patients with solitary lesions have an excellent prognosis. Whereas the outlook for patients with significant visceral involvement is not as good as those with bone lesions only, the outlook is by no means hopeless, as was once thought. A number of prognostic factors have been reviewed here. The most significant of these factors at the present time would appear to be age of onset of the disease, extent of involvement, the rapidity of progression of the disease, and, in particular, the presence or absence of dysfunction of such crucial organ systems as liver, lung, and hemopoietic system. Further studies of the significance of histologic features and immunologic findings are clearly needed to further our understanding of this disorder.
现在已经清楚,在过去几年里,朗格汉斯细胞组织细胞增多症患儿的预后有了显著改善。诚然,患有孤立性病变的患者预后极佳。虽然有明显内脏受累的患者的前景不如仅患有骨病变的患者,但绝不像曾经认为的那样毫无希望。这里回顾了一些预后因素。目前这些因素中最重要的似乎是疾病的发病年龄、受累程度、疾病进展的速度,特别是肝脏、肺和造血系统等关键器官系统是否存在功能障碍。显然需要进一步研究组织学特征和免疫学发现的意义,以加深我们对这种疾病的理解。