血嗜體貪食淋巴組織球增生症（HLH）是一種罕見且危及生命的綜合征，其特徵為過度炎性反應。成人HLH的相關數據有限。在本國家級的回顧性隊列研究中，我們分析了2006年10月1日至2019年12月31日期間收集的美國全國住院樣本數據庫的數據。使用國際疾病分類（ICD）代碼，我們確定了所有以HLH診斷被非選擇性入院的成年患者。我們描述了人口特徵、觸發因素和相關條件。使用連接點模型分析了診斷、治療和住院死亡的趨勢。使用多變量邏輯回歸模型比較了住院死亡率，並根據人口特徵和相關條件進行了調整。最後，我們描述了資源利用結果，包括住院費用和住院天數。我們確定了16,136例非選擇性成年HLH入院。人口金字塔顯示有兩個高峰分布，分別在年輕成年人（16-30歲）和老年成年人（56-70歲）。連接點回歸分析顯示，在研究期間內每10萬入院中HLH發生率顯著增加（平均每年百分比改變[APC] = 25.3％，p <0.0001），住院死亡率無顯著變化（斜率=-0.01，p = 0.95）或住院HLH治療施行率無顯著變化（斜率= 0.46，p = 0.20）。最常見的相關條件是惡性腫瘤（4953次[30.7％]）、感染（3913次[24.3％]）、自身免疫疾病（3362次[20.8％]）、器官移植狀態（639次[4％]）和先天免疫缺陷綜合徵（399次[2.5％]）。住院死亡率在老年人和男性中較高。此外，先天免疫缺陷綜合徵的住院死亡率最高（死亡率31.1％，調整后OR 2.36 [1.56-3.59]），其次是惡性腫瘤（死亡率28.4％，調整后OR 1.80 [1.46-2.22]）、感染（死亡率21.4％，調整后OR 1.33 [1.10-1.62]）、其他/無觸發因素（死亡率13.6％，調整后OR 0.73 [0.58-0.92]）、自身免疫疾病（死亡率13％，調整后OR 0.72 [0.57-0.92]）和器官移植后狀態（死亡率14.1％，調整后OR 0.64 [0.43-0.97]）。總體平均住院天數是14.3 ± 13.9天，平均住院費用為$54,900 ± 59,800。我們提供了對美國成人HLH負擔的見解。發病率持續增加，結果仍然不樂觀。這表明需要制定針對成人HLH的更新診斷和治療方案。無。© 2023作者

Haemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening syndrome characterized by an excessive inflammatory response. Limited data exist on adult HLH.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.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.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.None.© 2023 The Author(s).