Purpose

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).

Method

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.

Results

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 × 10⁹ /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.

Conclusion

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.

中文翻译：

---

基于血液的炎症生物标志物在继发性噬血细胞淋巴组织细胞增生症中的预后价值。

目的

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活化。本研究旨在探讨继发性HLH（sHLH）的可行预后因素。

方法

我们根据 HLH-2004 方案对 2016 年 1 月至 2019 年 5 月的 179 例新诊断 sHLH 患者进行回顾性分析。审查了基线特征和实验室结果。

结果

所有患者的中位年龄为 53 岁，男女之比为 1.45。HLH 的最常见原因是恶性肿瘤。在 179 名患者中，48.6% 有 EBV 感染，92.8% 有血细胞减少症（至少 2 个谱系），60.3% 有低纤维蛋白原血症，43.0% 有高甘油三酯血症（≥ 3 mmol/L），99.4% 有高铁蛋白，97.8% 有发热，72.1% 有脾肿大，72.6% 有噬血细胞。至于他们的预后，122名患者死亡；中位生存期为 88 天，2 年生存率为 26.72%。单变量分析证实中性粒细胞与淋巴细胞比 ˃ 2.53，淋巴细胞与单核细胞比 (LMR) ≤ 4.43，血小板与淋巴细胞比 ˃ 227.27，红细胞分布宽度 ˃ 14.6，红细胞分布宽度与血小板比率 (RPR) > 0.33，EBV 感染，血小板 ≤ 34 × 10 ⁹/L、纤维蛋白原≤ 1.34 g/L、碱性磷酸酶˃ 182.4 U/L、腺苷脱氨酶˃ 69.2 U/L 和铁蛋白˃ 2318 ng/mL 与较差的存活率相关。在多变量模型中，LMR、RPR 和铁蛋白被视为三个独立因素。

结论

一些基于血液的炎症标志物可以轻松且廉价地检测到，与 HLH 患者的 OS 显着相关。LMR和RPR优于NLR、PLR、RDW，可以用来预测HLH患者的OS。