Pediatrics Research Institute of Hunan Province, Hunan Children's Hospital, University of South China, Changsha, China.
Department of Pediatric Intensive Care Unit, Hunan Children's Hospital, University of South China, Changsha, China.
Ann Hematol. 2020 May;99(5):963-971. doi: 10.1007/s00277-020-03980-0. Epub 2020 Mar 27.
Patients with hemophagocytic lymphohistiocytosis (HLH) have high risk of early mortality. The purpose of this study was to test the hypothesis that the elevated level of serum myoglobin among patients with HLH is associated with disease severity and increased risk of mortality. We retrospectively investigated the serum myoglobin levels from 155 pediatric patients diagnosed with HLH in the Hunan Children's Hospital, China. The levels of myoglobin and creatine kinase at hospital admission among non-survivors and survivors were compared. The myoglobin level was dichotomized for the estimation of hazard ratio (HR) for mortality. Patients who died within 7 and 30 days of hospitalization had significantly higher myoglobin levels than did survivors (p < 0.05). The myoglobin level was negatively associated with the days of survival among non-survivors (Spearman correlation coefficient = - 0.29, p = 0.04). An elevated myoglobin level (> 90 ng/mL) was significantly associated with increased mortality (unadjusted HR = 2.66, 95%CI: 1.41, 5.00, p = 0.0024) and persisted after adjusting for age, Epstein-Barr virus infection, admission department, acute kidney injury, myocardial damage, and shock. In conclusion, an elevated serum myoglobin level was associated with increased risk of early death among pediatric patients with HLH, suggesting the potential of myoglobin to be used as a reference indicator for monitoring and managing of HLH.
噬血细胞性淋巴组织细胞增生症(HLH)患者有早期死亡的高风险。本研究旨在检验以下假说,即 HLH 患者血清肌红蛋白水平升高与疾病严重程度和死亡率增加相关。我们回顾性调查了中国湖南儿童医院 155 例诊断为 HLH 的儿科患者的血清肌红蛋白水平。比较了非幸存者和幸存者入院时肌红蛋白和肌酸激酶的水平。将肌红蛋白水平分为两部分,以估计死亡率的危险比(HR)。住院 7 天和 30 天内死亡的患者肌红蛋白水平明显高于幸存者(p<0.05)。非幸存者的肌红蛋白水平与生存天数呈负相关(Spearman 相关系数=-0.29,p=0.04)。较高的肌红蛋白水平(>90ng/mL)与死亡率增加显著相关(未调整的 HR=2.66,95%CI:1.41,5.00,p=0.0024),并且在调整年龄、EB 病毒感染、入院科室、急性肾损伤、心肌损伤和休克后仍然存在。总之,血清肌红蛋白水平升高与儿科 HLH 患者早期死亡风险增加相关,提示肌红蛋白可能作为监测和管理 HLH 的参考指标。