CAJ | 학술논문

目的探讨噬血细胞综合征(hemophagocytic syndrome,HPS)患儿诊断时的血清学指标与死亡的关系及对死亡的早期预测价值.方法采用回顾性病例对照研究方法,对2005年7月至2012年7月广州市妇女儿童医疗中心收治的108例HPS患儿血清学、病理学改变及预后资料进行系统分析.按随访患儿的生存情况分为存活组和死亡组,应用COX模型对可能与HPS死亡相关的危险因素进行分析；应用决策树探讨各指标对HPS死亡的预测价值.结果 108例HPS患儿中,死亡33例,病死率30.6％,且90.3％在发病后8周内死亡.多因素分析显示白细胞＜5×109/L(HR =9.08,95％ CI3.07 ～26.87)、血红蛋白＜80 g/L(HR =6.15,95％ CI 1.68 ～ 22.49)、白蛋白＜28g/L(HR=4.63,95％CI 1.12 ～7.39)、铁蛋白＞1 100 μg/L(HR =3.05,95％ CI 1.28 ～ 16.75)、甘油三酯≥4 mmol/L(HR=2.88,95％CI 1.51 ～8.60)、凝血酶原时间≥16s(HR=3.60,95％CI 1.28 ～7.24)和发热持续2周以上(HR =5.39,95％CI 1.97～14.66)是造成HPS患儿死亡的独立危险因子.决策树分析显示白细胞＜5×109/L合并血红蛋白＜80 g/L的情况下患儿死亡概率达100％;即使在白细胞≥5×109/L的情况下,若合并发热持续2周以上且总胆固醇≥4 mmol/L,死亡概率亦达66.7％.结论发病后最初8周是治疗HPS的关键期,HPS患儿死亡与多种因素相关,合并白细胞＜5×109/L且血红蛋白＜80 g/L时,病情极其凶险,及早诊断并采取有针对性的治疗对降低HPS患儿病死率至关重要.
목적 탐토서혈세포종합정(hemophagocytic syndrome,HPS)환인진단시적혈청학지표여사망적관계급대사망적조기예측개치.방법 채용회고성병례대조연구방법,대2005년7월지2012년7월엄주시부녀인동의료중심수치적108례HPS환인혈청학、병이학개변급예후자료진행계통분석.안수방환인적생존정황분위존활조화사망조,응용COX모형대가능여HPS사망상관적위험인소진행분석；응용결책수탐토각지표대HPS사망적예측개치.결과 108례HPS환인중,사망33례,병사솔30.6％,차90.3％재발병후8주내사망.다인소분석현시백세포＜5×109/L(HR =9.08,95％ CI3.07 ～26.87)、혈홍단백＜80 g/L(HR =6.15,95％ CI 1.68 ～ 22.49)、백단백＜28g/L(HR=4.63,95％CI 1.12 ～7.39)、철단백＞1 100 μg/L(HR =3.05,95％ CI 1.28 ～ 16.75)、감유삼지≥4 mmol/L(HR=2.88,95％CI 1.51 ～8.60)、응혈매원시간≥16s(HR=3.60,95％CI 1.28 ～7.24)화발열지속2주이상(HR =5.39,95％CI 1.97～14.66)시조성HPS환인사망적독립위험인자.결책수분석현시백세포＜5×109/L합병혈홍단백＜80 g/L적정황하환인사망개솔체100％;즉사재백세포≥5×109/L적정황하,약합병발열지속2주이상차총담고순≥4 mmol/L,사망개솔역체66.7％.결론 발병후최초8주시치료HPS적관건기,HPS환인사망여다충인소상관,합병백세포＜5×109/L차혈홍단백＜80 g/L시,병정겁기흉험,급조진단병채취유침대성적치료대강저HPS환인병사솔지관중요.
Objective To investigate the association with death for serum parameters at the time of diagnosis and its value in predicting the death in infants with hemophagocytic syndrome (HPS).Methods A retrospective case-control study was conducted on 108 children with HPS who were admitted to our center between July 2005 and July 2012.For each patient,demographic,laboratory data and outcome information were collected.The patients were divided into death and surviving groups based on the follow-up results.The relation between serum markers and death was examined using the COX proportional hazards model and decision tree.Results Of 108 infants with HPS,33 died corresponding to a fatality rate of 30.6％ and 90.3％ of deaths occurred within 8 weeks after diagnosis.Following features were significantly associated with death:white blood cells (WBC) ＜5 x 109/L (HR =9.08,95％ CI 3.07 ～ 26.87),hemoglobin ＜80 g/L (HR =6.15,95％ CI 1.68 ～ 22.49),albumin ＜ 28 g/L (HR =4.63,95％ CI 1.12 ～ 7.39),serum ferritin ＞ 1 100 μg/L (HR =3.05,95％ CI 1.28 ～ 16.75),trigeminal ganglion ≥4 mmol/L (HR =2.88,95％ CI 1.51 ～ 8.60),and prothromin time ≥ 16 s (HR =3.60,95 ％ CI 1.28 ～ 7.24),and fever for more than 2 weeks (HR =5.39,95％ CI 1.97 ～ 14.66).Decision tree demonstrated that the probability of death was as high as 100％ for infants with WBC ＜5 x 109/L and hemoglobin ＜ 80 g/L.The odds of dying was still 66.7％ for infants who had WBC≥5 × 109/L but reported trigeminal ganglion ≥4 mmol/L after having fever for more than 2 weeks.Conclusion The first 8 weeks after the onset of HPS is the critical period of treatment.There are several easily available serum predictors of early mortality in HPS infants,particularly the WBC and hemoglobin level,which may help guide treatment decisions.