[The Clinical and Laboratory Characteristics of Adult Langerhans Cell Histiocytosis Patients and the Influencing Factors of Prognosis]

To observe the clinical and laboratory characteristics of adult Langerhans cell histiocytosis(LCH) patients and to analyze the influencing factors of its prognosis.The clinical and laboratory charac-teristics of 38 adult LCH patients treated in our hospital from January 2010 to August 2019 were retrospective analyzed, and the clinical prognosis of the patients was analyzed.The median age of 38 patients was 41 (21-65) years old, and the ratio of male and female was about 2∶1. Among 38 patients, 44.7% (17/38) were involved in multiple systems, and 31.6% (12/38) were involved in high-risk organs (including liver, lung, hematopoietic system or spleen). The bone involvement was the most common (21/38, 55.3%), and the most common clinical symptom was pain (19/38, 50.0%). The result of laboratory showed that anemia (4/38，10.5%), thrombocytopenia (1/38，2.6%), neutropenia (2/38，5.3%), lymphopenia (6/38，15.8%), monocytosis (11/38，28.9%), C-reactive protein increasing (6/21，28.6%), erythrocyte sedimentation rate increasing (10/18, 55.3%), and ferritin protein increasing (9/17, 55.3%). The median follow-up time was 53 months, and a total of 5 patients were died. The 10-year overall survival rate of patients with single-system involvement was 100%, which was significantly higher than that of patients with multiple-system involvement (70.1%) (P=0.0078). The prognosis of patients without risk-organ involvement was better than that of patients with risk-organ involvement (10-year overall survival rate: 100% vs 60.6%) (P=0.0007). Further analysis showed that in addition to multiple-system involvement and risk-organ involvement, the increase of peripheral blood monocyte cells and the increase of ferritin protein were also associated with poorer prognosis of the patients.The multiple system involve-ment and risk-organ involvement, the increasing of monocyte cells and the increasing of ferritin protein were the independent risk factors of adult LCH patients.成人朗格汉斯组织细胞增多症患者的临床实验特征以及影响预后的因素分析.观察分析成人朗格汉斯组织细胞增生症（LCH）患者的临床实验特征及预后因素.回顾性分析2010年1月至2019年8月就诊于本院且经组织病理学确诊的成人LCH患者38例的临床特征和实验室检查特征，并对患者的临床预后进行深入分析.38例LCH患者中位年龄为41（21-65岁）岁，男女比约为2∶1。38例LCH患者中，出现多系统累及占44.7%，31.6%的患者具有危险器官(肝脏、肺、造血系统或脾脏)累及，最常见受累器官为骨骼（21/38，55.3%），最常见的临床症状为疼痛（19/38，50.0%）。实验室检查显示，贫血、血小板减少、中性粒细胞减少、淋巴细胞减少、单核细胞增多、C反应蛋白升高、血沉升高以及铁蛋白升高的发生率分别为10.5%、2.6%、5.3%、15.8%、28.9%、28.6%（6/21）、55.3%（10/18）及55.3%（9/17）。中位随访时间为53个月，共5例患者死亡。单系统受累患者10年总生存（OS）率为100%，显著优于多系统受累患者（70.1%）（P=0.0078）。无危险器官受累患者的预后显著优于有危险器官受累的患者，患者10年OS率分别为100%和60.6%（P=0.0007）。进一步分析发现，除多系统受累与危险器官受累为不良预后因素外，初诊时外周血单核细胞增多以及铁蛋白升高与较差的预后显著相关.多系统受累与危险器官受累、初诊时外周血单核细胞增多以及铁蛋白升高是成人LCH患者重要的预后不良因素.