成人嗜血综合征急诊激素治疗对诊断和预后的影响

Adult hemophagocytic syndrome in emergency department setting:the implications of hormone therapy on diagnosis and prognosis

目的探讨急诊激素治疗对成人嗜血细胞综合征(hemophagocytic syndrome,HPS)患者临床诊断建立和临床预后的影响。方法回顾性收集2019年9月至2021年8月北京大学人民医院急诊科符合研究要求的拟诊HPS患者共34例。依据院内死亡情况将患者分为死亡组和生存组,依据激素应用情况将患者分为提前激素治疗组和标准激素治疗组。提前激素治疗组患者依据满足4项HLH-2004诊断标准时的情况分为常规项目组和非常规项目组。收集患者入院时血常规、生化全项、凝血分析,血清铁蛋白,NK细胞活性,sCD25水平,外周血涂片、骨髓穿刺、腹部超声/CT等临床指标,并在5~7 d后复查血常规、生化全项及凝血分析等临床指标,并进行统计学分析。结果①死亡组中HPS患者年龄较大,APACHEⅡ评分及SOFA评分较高,总胆红素较高,血清白蛋白较低。②单因素Logistic分析显示年龄(OR=1.098,CI:1.019~1.183,P=0.014),APACHEⅡ评分(OR=1.144,CI:1.017~1.285,P=0.024),SOFA评分(OR=1.441,CI:1.079~1.925,P=0.013)与患者死亡风险相关,多因素Logistic分析显示年龄(OR=1.099,CI:1.014~1.190,P=0.021)与患者死亡风险相关。提前激素治疗与患者临床预后无明显相关。Kaplan-Meier生存曲线显示:提前激素治疗组与标准激素治疗组相比,患者60 d生存率差异无统计学意义。③提前激素治疗后甘油三酯水平仍升高,满足HLH-2004诊断标准项目数明显增加,全部患者均满足Hscore>169分标准,3例患者未满足至少5项HLH-2004诊断标准,占提前激素治疗总病例数的16.7%。④满足4项HLH-2004诊断标准时开始激素治疗可以降低住院时间,治疗后5~7 d患者凝血酶原时间和部分活化凝血酶原时间更接近正常水平,提前激素治疗对治疗应答、院内死亡风险无明显影响。常规项目组和非常规项目组相比:APACHEⅡ评分、SOFA评分、建立诊断、治疗应答、临床预后及激素治疗后相关临床指标差异无统计学意义。结论提前激素治疗对患者HPS的临床诊断建立、治疗应答、院内病死率和60 d生存率无明显影响,可以更快纠正凝血功能障碍,有效减少住院时间。急诊医生高度怀疑患者HPS诊断时,可以考虑更早的(满足4项HLH-2004诊断标准)开始激素治疗。

Objective To assess the implications of hormone therapy on confirmation of clinical diagnosis and prognosis of adult hemophagocytic syndrome(HPS)in the emergency department setting.Methods The eligible 34 patients admitted with suspected HPS in the Emergency Department of Peking University People's Hospital from September 2019 to August 2021 were respectively collected.The patients were divided into the death group and survival group according to the prognosis and divided into early hormone therapy group and standard hormone therapy group according to the timing of hormone application.Patients in the early hormone therapy group were divided into the routine 4 criteria group and non-routine 4 criteria group according to the conditions of meeting the four HLH-2004 diagnostic criteria.Medical records of the following were collected and statistically analyzed:complete blood count,blood biochemical index,coagulation function,serum ferritin,NK cell activity,sCD25 level,peripheral blood smear,bone marrow biopsy,abdominal ultrasound scan,and abdominal CT on admission,and recheck the clinical indicators such as blood count,blood biochemical index and blood coagulation dunction 5-7 days later.Results①Patients from the death group were older,with higher APACHEⅡscores and SOFA scores,higher total bilirubin,and lower serum albumin.②Univariate Logistic analysis showed age(OR=1.098,CI:1.019-1.183,P=0.014),APACHEⅡscore(OR=1.144,CI:1.017-1.285,P=0.024),SOFA score(OR=1.441,CI:1.079-1.925,P=0.013)were associated with the risk of death.Multivariate Logistic analysis showed that age(OR=1.099,CI:1.014-1.190,P=0.021)was associated with the risk of death.There was no significant correlation between early hormone therapy and clinical prognosis.Kaplan-Meier survival curves showed that there was no difference in the 60-day survival rate between the early hormone therapy group and the standard hormone therapy group.③The level of triglyceride still increased after early hormone therapy,and the number of indexes meeting the diagnostic criteria of HLH-2004 increased significantly.All patients met the criteria of Hscore>169,and 3 patients did not meet at least 5 diagnostic criteria of HLH-2004,accounting for 16.7%of the total cases of early hormone therapy.④Starting hormone therapy when the four HLH-2004 diagnostic criteria were met could reduce the length of hospital stay.Prothrombin time and activited partial thomboplastin time were closer to normal levels in patients 5-7 days after treatment.Early hormone therapy had no significant effect on treatment response and inhospital death risk.There were no significant differences in APACHEⅡscore,SOFA score,confirmation of diagnosis,treatment response,clinical prognosis,and related clinical indicators after hormone therapy between the routine 4 criteria and non-routine 4 criteria groups.Conclusions Initiation of early hormone therapy has no significant effect on the confirmation of clinical diagnosis,treatment response,in-hospital mortality,and 60-day survival rate of patients with HPS,and can quickly correct coagulation dysfunction and effectively reduce the length of hospital stay.An earlier start of hormonal therapy(meeting the four HLH-2004 diagnostic criteria)may be considered by the emergency physician when a patient is highly suspected of HPS diagnosis.