La Marle Solène, Richard-Colmant Gaëlle, Fauvernier Mathieu, Ghesquières Hervé, Hot Arnaud, Sève Pascal, Jamilloux Yvan
Département de Médecine Interne, Hôpital de la Croix Rousse-Hospices Civils de Lyon, Université Claude Bernard-Lyon 1, 69002 Lyon, France.
Département de Biostatistique-Bioinformatique, Pôle Santé Publique, Hospices Civils de Lyon, Université Claude Bernard-Lyon 1, 69000 Lyon, France.
J Clin Med. 2023 Feb 20;12(4):1696. doi: 10.3390/jcm12041696.
Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome with an overall mortality rate of 40%. A multiple-cause-of-death analysis allows for the characterization of mortality and associated causes over an extended period. Death certificates, collected between 2000 and 2016 by the French Epidemiological Centre for the Medical Causes of Death (CepiDC, Inserm), containing the ICD10 codes for HLH (D76.1/2), were used to calculate HLH-related mortality rates and to compare them with the general population (observed/expected ratios, O/E). HLH was mentioned in 2072 death certificates as the underlying cause of death (UCD, = 232) or as a non-underlying cause of death (NUCD, = 1840). The mean age at death was 62.4 years. The age-standardized mortality rate was 1.93/million person-years and increased over the study period. When HLH was an NUCD, the most frequently associated UCDs were hematological diseases (42%), infections (39.4%), and solid tumors (10.4%). As compared to the general population, HLH decedents were more likely to have associated CMV infections or hematological diseases. The increase in mean age at death over the study period indicates progress in diagnostic and therapeutic management. This study suggests that the prognosis of HLH may be at least partially related to coexisting infections and hematological malignancies (either as causes of HLH or as complications).
噬血细胞性淋巴组织细胞增生症（HLH）是一种严重的高炎症综合征，总体死亡率为40%。多死因分析有助于在较长时期内对死亡率及相关病因进行特征描述。法国医学死因流行病学中心（CepiDC，法国国家健康与医学研究院）在2000年至2016年期间收集的死亡证明，包含HLH的ICD10编码（D76.1/2），用于计算HLH相关死亡率，并将其与普通人群进行比较（观察/预期比率，O/E）。在2072份死亡证明中，HLH被提及为根本死因（UCD，=232）或非根本死因（NUCD，=1840）。平均死亡年龄为62.4岁。年龄标准化死亡率为每百万人年1.93例，且在研究期间有所上升。当HLH为非根本死因时，最常相关的根本死因是血液系统疾病（42%）、感染（39.4%）和实体瘤（10.4%）。与普通人群相比，HLH死者更有可能伴有巨细胞病毒感染或血液系统疾病。研究期间平均死亡年龄的增加表明诊断和治疗管理方面取得了进展。这项研究表明，HLH的预后可能至少部分与并存感染和血液系统恶性肿瘤有关（无论是作为HLH的病因还是并发症）。
