Jubran Rima F, Marachelian Araz, Dorey Frederick, Malogolowkin Marcio
Keck School of Medicine, University of Southern California, Division of Pediatric Hematology/Oncology, Children's Hospital Los Angeles, USA.
Pediatr Blood Cancer. 2005 Jul;45(1):37-42. doi: 10.1002/pbc.20364.
Our goal was to examine the clinical course of patients with Langerhans cell histiocytosis (LCH), with a special emphasis on bone disease and to attempt to identify and examine the factors that may predict reactivation and overall prognosis.
We conducted a retrospective chart review of 132 consecutive pediatric patients treated at Children's Hospital Los Angeles for LCH from 1984 to 2001.
The risk for reactivation after initial management is significantly higher for patients with multiple bone and those with multiple organ involvement as compared with patients who had a single bone lesion (hazard ratios are 7.1 and 11.6). Patients younger than 1 year in the multiple organ group have an increased risk of death at 2 years when compared with the older patients in that group (hazard ration = 6.2, P = 0.022). Endocrine abnormalities were seen in 20% and 7.5% of patients with or without skull lesions respectively.
Patients with LCH involving only the bones have a significantly better outcome than those with other organ involvement. Patients with multiple organ involvement who are less than 1 year of age are at high risk of death and should be approached more aggressively upfront.
我们的目标是研究朗格汉斯细胞组织细胞增多症（LCH）患者的临床病程，特别关注骨病，并试图识别和研究可能预测疾病复发及总体预后的因素。
我们对1984年至2001年在洛杉矶儿童医院接受治疗的132例连续性儿科LCH患者进行了回顾性病历审查。
与仅有单个骨病变的患者相比，多骨受累和多器官受累患者初始治疗后疾病复发的风险显著更高（风险比分别为7.1和11.6）。多器官受累组中1岁以下的患者与该组中年龄较大的患者相比，2年时死亡风险增加（风险比=6.2，P=0.022）。有或无颅骨病变的患者中，内分泌异常分别见于20%和7.5%的患者。
仅累及骨骼的LCH患者的预后明显优于其他器官受累的患者。年龄小于1岁的多器官受累患者死亡风险高，应在治疗初期采取更积极的治疗措施。
