■噬血细胞性淋巴组织细胞增生症(HLH)是一种罕见且危及生命的综合征,其特征是过度的炎症反应。关于成人HLH的数据有限。
■在这个国家,回顾性队列研究,我们分析了2006年10月1日至2019年12月31日收集的美国全国住院患者样本数据库的数据.使用国际疾病分类(ICD)代码,我们确定了所有非选择性入院并诊断为HLH的成年患者.我们描述了人口统计特征,触发器,和相关条件。诊断趋势,治疗,和院内死亡率使用连接点模型进行分析.使用多变量逻辑回归模型比较了住院死亡率,该模型根据人口统计学特征和相关条件进行了调整。最后,我们描述了资源利用结局,包括住院费用和住院时间.
■我们确定了16,136名非选择性成人HLH入学。人口金字塔呈双峰分布,在年轻人(16-30岁)和老年人(56-70岁)的高峰。Joinpoint回归分析显示,在研究期间,每100,000例入院的HLH发病率显着增加(平均年变化百分比[APC]=25.3%,p<0.0001),住院死亡率(斜率=-0.01;p=0.95)或住院HLH治疗(斜率=0.46,p=0.20)无显著变化.最常见的相关疾病是恶性肿瘤(4953例入院[30.7%]),感染(3913例入院[24.3%]),自身免疫性疾病(3362例入院[20.8%]),器官移植状况(639例入院[4%]),和先天性免疫缺陷综合征(399例入院[2.5%])。老年人和男性的住院死亡率较高。此外,先天性免疫缺陷综合征的住院死亡率最差(死亡率为31.1%,调整后OR2.36[1.56-3.59]),其次是恶性肿瘤(死亡率28.4%,调整后OR1.80[1.46-2.22]),感染(死亡率21.4%,调整后OR1.33[1.10-1.62]),其他/无触发因素(死亡率13.6%,调整后OR0.73[0.58-0.92]),自身免疫性(死亡率13%,调整后OR0.72[0.57-0.92]),和器官移植后状况(死亡率14.1%,调整后OR0.64[0.43-0.97])。总体平均住院时间为14.3±13.9天,平均住院费用为$54,900±59,800。
■我们提供了对美国成人HLH负担的见解。发病率一直在增加,结果仍然令人沮丧。这表明越来越需要开发针对成人HLH的更新的诊断和治疗方案。
■无。
UNASSIGNED: Haemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening syndrome characterized by an excessive inflammatory response. Limited data exist on adult HLH.
UNASSIGNED: In this national, retrospective cohort study, we analysed data from the US National Inpatient Sample database collected between October 1, 2006 and December 31, 2019. Using the International Classification of Diseases (ICD) codes, we identified all adult patients who were admitted non-electively with the diagnosis of HLH. We described demographic characteristics, triggers, and associated conditions. Trends of diagnosis, treatment, and in-hospital mortality were analysed using joinpoint models. In-hospital mortality rates were compared using multivariable logistic regression models that adjusted for demographic characteristics and associated conditions. Finally, we described resource utilization outcomes including cost of hospitalization and length of stay.
UNASSIGNED: We identified 16,136 non-elective adult HLH admissions. The population pyramid showed a bimodal distribution, with peaks in young adults (16-30 years) and older adults (56-70 years). Joinpoint regression analysis revealed a significant increase in HLH incidence per 100,000 admissions over the study period (Average Annual Percent Change [APC] = 25.3%, p < 0.0001), and no significant change in rates of in-hospital mortality (slope = -0.01; p = 0.95) or administration of in-hospital HLH treatment (slope = 0.46, p = 0.20). The most common associated conditions were malignancy (4953 admissions [30.7%]), infections (3913 admissions [24.3%]), autoimmune conditions (3362 admissions [20.8%]), organ transplant status (639 admissions [4%]), and congenital immunodeficiency syndromes (399 admissions [2.5%]). In-hospital mortality was higher in older adults and males. Furthermore, Congenital immunodeficiency syndromes had the worst in-hospital mortality rate (mortality rate 31.1%, adjusted OR 2.36 [1.56-3.59]), followed by malignancies (mortality rate 28.4%, adjusted OR 1.80 [1.46-2.22]), infections (mortality rate 21.4%, adjusted OR 1.33 [1.10-1.62]), other/no trigger (mortality rate 13.6%, adjusted OR 0.73 [0.58-0.92]), autoimmune (mortality rate 13%, adjusted OR 0.72 [0.57-0.92]), and post-organ transplant status (mortality rate 14.1%, adjusted OR 0.64 [0.43-0.97]). The overall mean length of stay was 14.3 ± 13.9 days, and the mean cost of hospitalization was $54,900 ± 59,800.
UNASSIGNED: We provide insight into the burden of adult HLH in the USA. The incidence has been increasing and the outcomes remain dismal. This signifies the growing need for the development of updated diagnosis and treatment protocols that are specific to adult HLH.
UNASSIGNED: None.