噬血细胞综合征临床病因及预后研究:56例并文献复习

Clinical etiology and prognosis of hemophagocytic lymphohistiocytosis:56 cases and literature review

目的:分析噬血细胞综合征(hemophagocytic lymphohistiocytosis,HLH)患者的临床病因及预后特点。方法:回顾56例HLH患者的临床资料,分析其一般资料、临床特点、临床病因的频率及分布,应用Kaplan-Meier法绘制生存曲线并进行logrank检验以分析生存时间和比较预后差异。结果:56例HLH患者临床病因以淋巴瘤最常见(32.14%),其次为感染(30.36%),其中EB病毒(Epstein-Barr virus,EBV)感染最常见(16.07%),自身免疫性疾病占17.86%,原发性HLH占12.50%。56例HLH患者第1、3、6、9、12、18个月的生存率分别为73.2%、50.0%、44.6%、41.1%、39.3%及30.4%,各病因组生存曲线不同,其中自身免疫性疾病组生存率明显高于其他组,EBV-HLH患者生存率低于未感染者,未使用依托泊苷的患者生存率低于使用者,使用芦可替尼的患者与未使用者生存率无统计学差异。结论:淋巴瘤为HLH的最常见病因,不同病因的HLH发生率及生存率不同,EBV-HLH患者生存率低,预后差。依托泊苷可使患者生存受益,但患者并未从芦可替尼中受益。

Objective:To explore the clinical etiology and prognosis of hemophagocytic lymphohistiocytosis(HLH). Methods:Clinical information of 56 patients with HLH was reviewed. The frequency and distribution of general information,clinical characteristic and clinical pathogen were analyzed. Survival curve by Kaplan-Meier method and log-rank test were utilized for analysis of survival time and comparison of prognosis respectively. Results:Among the 56 cases of HLH,lymphoma was the most common cause(32.14%),followed by infection(30.36%),among which EBV infection was the most common(16.07%). Autoimmune diseases accounted for 17.86%,and primary HLH accounted for 12.50%. The survival rates of 56 patients with HLH in the 1st,3rd,6th,9th,12th,18th month were73.2%,50.0%,44.6%,41.1%,39.3% and 30.4% respectively. The survival curves of each etiology group were different. The survival rate of autoimmune disease group were significantly higher than that of other groups. EBV-HLH patients had lower survival rate than uninfected patients. Patients without etoposide had lower survival rate than users,and there was no significant difference in the survival rate between patients who used ruxolitinib and those who did not. Conclusion:Lymphoma is the most common cause of HLH. The incidence and survival rate of HLH are different with different causes. EBV-HLH patients have low survival rates and poor prognosis.The use of etoposide can improve the survival rate of patients,but ruxolitinib can’t.