恶性相关的血吞噬细胞淋巴组织细胞增生症（mal-HLH）是一种预后不良的严重高炎症综合征。我们评估了瑞典的mal-HLH病例，对与人口的发病率、临床特征和生存率相关的因素进行了研究。使用1997-2018年的瑞典患者登记册，我们确定了307名成年人（≥18岁）和9名儿童（男性209名，女性107名，p<0.001），这些患者同时存在HLH相关诊断和恶性疾病，根据ICD-10疾病代码进行诊断，相当于每年每10万成年人发病率为0.19/10万（对于整个人群为0.15/10万）；由1997-2007年的0.026增加到2008-2018年的0.34（p<0.001）。在最近的7年期间（2012-2018年），发病率为每年每10万成年人0.45（n=246）。该发病率在瑞典的六个卫生保健区之间存在差异，从0.18到0.71（斯德哥尔摩地区）每10万成年人不等（p<0.001），可能是由于对该病不同程度的认识。mal-HLH在所有血液恶性肿瘤中的报道比例为0.6%，最高比例（2.8%）出现在25-29岁男性中。在316例患者中，1个月生存概率（可能代表HLH发作）从1997-2007年的52%（置信区间（CI）40-63）增加到2008-2018年的71%（CI=65-76），而2年生存率仍然较低（25%，20-30%）。总体而言，52%为淋巴瘤；29%为白血病；8%为其他血液恶性肿瘤；11%为实体肿瘤。男性决定laG的发病率要高于女性，这也考虑到男性在血液恶性肿瘤中的过度代表性（p=0.0012）。值得注意的是，对1997-2011年的医疗文件进行验证后发现，HLH诊断存在13%的过度报告。我们得出结论：每年mal-HLH的发病率在1997-2018年间增加了十倍，2012-2018年至少为每10万成年人0.71的发病率，即在估计HLH过度报告的基础上为每10万成年人0.62的发病率，并且早期生存率显著提高，这可能是由于对该病的认知提高以及针对HLH的治疗更加精细化。本文版权归2023年美国血液学学会所有。

Malignancy-associated hemophagocytic lymphohistiocytosis (mal-HLH) is a severe hyperinflammatory syndrome with poor prognosis. We evaluated mal-HLH in Sweden with regard to population-based incidence, clinical features, and survival. Using the Swedish Patient Registry for 1997-2018, we identified 307 adults (≥18-years) and 9 children (209 males, 107 females; p<0.001), with both an HLH-related diagnosis and malignant disease according to ICD-10, corresponding to 0.19/100,000 adults/year (0.15/100,000 for the entire population); increasing from 0.026 1997-2007 to 0.34 2008-2018 (p<0.001). The most recent 7-year period (2012-2018), the incidence was 0.45/100,000 adults/year (n=246). This incidence varied between the six health care regions in Sweden, from 0.18 to 0.71 (Region Stockholm) per 100,000 adults/year (p<0.001), likely due to variable awareness. Mal-HLH was reported in 0.6% of all hematological malignancies with highest proportion (2.8%) in males aged 25-29 years. Among all 316 patients, 1-month probability of survival, likely representing the HLH-episode, increased significantly from 52% (confidence interval (CI) 40-63) 1997-2007 to 71% (CI=65-76) 2008-2018, while 2-year survival remained poor (25%; 20-30%). Altogether 52% were lymphomas; 29% leukemias; 8% other hematological malignancies; and 11% solid tumors. Men were more affected than females by mal-HLH, also taking the overrepresentation of men with hematological malignancies into account (p=0.0012). Notably, validation by review of medical files 1997-2011 revealed 13% over-reporting of the diagnosis HLH. We conclude that the annual mal-HLH incidence increased ten-fold 1997-2018, was at least 0.71/100,000 adults 2012-2018, i.e., 0.62/100,000 adults considering 13% estimated HLH over-reporting, and that early survival improved significantly, likely due to increased awareness and more HLH-directed therapy.Copyright © 2023 American Society of Hematology.