Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, 300 Guangzhou Road, Nanjing, 210029, China.
Department of Geriatrics, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital, 300 Guangzhou Road, Nanjing, 210029, China.
J Clin Immunol. 2020 Jul;40(5):718-728. doi: 10.1007/s10875-020-00801-x. Epub 2020 Jun 3.
Hemophagocytic lymphohistiocytosis (HLH) is a rare systematic immune disease manifested with excessive activation of lymphocytes and macrophages. This study was designed to explore the feasible prognostic factors of secondary HLH (sHLH).
We retrospectively analyzed 179 patients with newly diagnosed sHLH from January 2016 to May 2019 according to the HLH-2004 protocol. Baseline characteristics and laboratory results were reviewed.
The median age of all patients was 53 years, with a male/female ratio of 1.45. The commonest cause of HLH was malignancy. Of the 179 patients, 48.6% presented with Epstein-Barr virus (EBV) infection, 92.8% with hemocytopenia (at least 2 lineages), 60.3% with hypofibrinogenemia, 43.0% with hypertriglyceridemia (≥ 3 mmol/L), 99.4% with high ferritin, 97.8% with fever, 72.1% with splenomegaly, and 72.6% with hemophagocytosis. As to their prognosis, 122 patients died; the median survival was 88 days, with a 2-year survival rate of 26.72%. Univariate analysis confirmed neutrophil-to-lymphocyte ratio ˃ 2.53, lymphocyte-to-monocyte ratio (LMR) ≤ 4.43, platelet-to-lymphocyte ratio ˃ 227.27, red blood cell distribution width ˃ 14.6, red blood cell distribution width-to-platelet ratio (RPR) > 0.33, EBV infection, platelet ≤ 34 × 10 /L, fibrinogen ≤ 1.34 g/L, alkaline phosphatase ˃ 182.4 U/L, adenosine deaminase ˃ 69.2 U/L, and ferritin ˃ 2318 ng/mL were associated with an inferior survival. In a multivariate model, LMR, RPR, and ferritin were considered as three independent factors.
Some blood-based inflammatory markers, which can be easily and cheaply detected, are significantly associated with the OS of HLH patients. LMR and RPR, superior to NLR, PLR, RDW, can be taken to predict the OS of patients with HLH.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的系统性免疫疾病，表现为淋巴细胞和巨噬细胞过度激活。本研究旨在探讨继发性 HLH（sHLH）的可行预后因素。
我们根据 HLH-2004 方案回顾性分析了 2016 年 1 月至 2019 年 5 月期间确诊的 179 例新诊断 sHLH 患者。回顾了基线特征和实验室结果。
所有患者的中位年龄为 53 岁，男女比例为 1.45。HLH 的最常见病因是恶性肿瘤。179 例患者中，48.6%为 EBV 感染，92.8%为血细胞减少（至少 2 系），60.3%为低纤维蛋白原血症，43.0%为高甘油三酯血症（≥3mmol/L），99.4%为高铁蛋白血症，97.8%为发热，72.1%为脾肿大，72.6%为噬血细胞。就预后而言，122 例患者死亡；中位生存时间为 88 天，2 年生存率为 26.72%。单因素分析证实中性粒细胞与淋巴细胞比值＞2.53、淋巴细胞与单核细胞比值（LMR）≤4.43、血小板与淋巴细胞比值＞227.27、红细胞分布宽度＞14.6、红细胞分布宽度与血小板比值（RPR）＞0.33、EBV 感染、血小板≤34×10/L、纤维蛋白原≤1.34g/L、碱性磷酸酶＞182.4U/L、腺苷脱氨酶＞69.2U/L、铁蛋白＞2318ng/mL 与生存不良相关。多因素模型中，LMR、RPR 和铁蛋白被认为是三个独立因素。
一些基于血液的炎症标志物，这些标志物可以方便且廉价地检测到，与 HLH 患者的 OS 显著相关。LMR 和 RPR 优于 NLR、PLR、RDW，可用于预测 HLH 患者的 OS。
