Department of Pathology and Laboratory Medicine, Henry Ford Hospital, Detroit, MI, USA.
Department of Pathology and Immunology, Washington University School of Medicine, St. Louis, MO, USA.
Int J Lab Hematol. 2021 Oct;43(5):1024-1031. doi: 10.1111/ijlh.13489. Epub 2021 Feb 17.
Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome of pathologic immune activation. Most studies on adult HLH have evaluated prognostic factors for overall survival; factors predicting early mortality have not been sufficiently investigated.
This was a collaborative study between Henry Ford Hospital and Barnes-Jewish Hospital. We identified all adult HLH patients with at least 2 ferritin levels within 30 days from admission.
One-hundred twenty-four patients were identified. There were 77 males and 47 females; the median age at diagnosis was 48 years. Multivariate analysis showed that age (OR = 11.41; 95% CI:2.71-48.04; P = .001), hepatomegaly (OR = 15.68; 95% CI:3.24-75.96; P = .001), hyponatremia (OR = 5.94; 95% CI:1.76-20.1; P = .004), hypoalbuminemia (OR = 7.47; 95% CI:2.08-26.85; P = .002), and increasing ferritin levels (OR = 19.46; 95% CI:4.69-80.71; P < .001) were significant predictors of 30-day mortality. Patients with declining ferritin by more than 35% from the ferritin peak were more likely to survive the first 30 days of admission (OR = 4.33; 95% CI:1.04-18.1; P = .033). By risk stratifying our cohort, we identified changes in ferritin levels to be the most significant prognostic factor of 30-day mortality among other risk factors. Further investigating the prognostic utility of ferritin showed that increasing ferritin during the 1st week of admission (data available for 44 patients) was the only significant predictor of 30-day mortality.
To the best of our knowledge, this is the first study reporting changes in ferritin to be a predictor for early death in adult HLH. Changes in ferritin might be a useful indicator of adult HLH disease activity and early prognosis.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见的病理性免疫激活综合征。大多数关于成人 HLH 的研究都评估了总生存的预后因素；但尚未充分研究预测早期死亡率的因素。
这是亨利福特医院和巴恩斯犹太医院之间的合作研究。我们在入院后 30 天内至少有 2 次铁蛋白水平的情况下，确定了所有成年 HLH 患者。
共确定了 124 名患者。其中男 77 例，女 47 例；诊断时的中位年龄为 48 岁。多变量分析显示，年龄（OR=11.41；95%CI：2.71-48.04；P=0.001）、肝肿大（OR=15.68；95%CI：3.24-75.96；P=0.001）、低钠血症（OR=5.94；95%CI：1.76-20.1；P=0.004）、低白蛋白血症（OR=7.47；95%CI：2.08-26.85；P=0.002）和铁蛋白水平升高（OR=19.46；95%CI：4.69-80.71；P<0.001）是 30 天死亡率的显著预测因素。铁蛋白峰值下降超过 35%的患者更有可能在入院后 30 天内存活（OR=4.33；95%CI：1.04-18.1；P=0.033）。通过对我们的队列进行风险分层，我们发现铁蛋白水平的变化是其他危险因素中 30 天死亡率的最重要预后因素。进一步研究铁蛋白的预后应用价值表明，入院后第 1 周铁蛋白升高（可用于 44 名患者的数据）是 30 天死亡率的唯一显著预测因素。
据我们所知，这是第一项报告铁蛋白变化可预测成人 HLH 早期死亡的研究。铁蛋白的变化可能是成人 HLH 疾病活动和早期预后的有用指标。
