儿童EB病毒相关性噬血细胞综合征与传单临床特点比较

Clinical Characteristics Comparison of Children EB Virus Correlation Hemophagocytic Syndrome and Infectious Mononucleosis

目的通过分析比较儿童EB病毒相关性噬血细胞综合征(EBV-HPS)与传染性单核细胞增多症(IM)患者的临床表现及实验室特点,提高对EBV-HPS的认识,减少误诊,降低死亡率。方法回顾性分析45例EBV-HPS患者及同期收治的93例IM患者。EB-HPS组中,男24例,女21例,男女比例为1.14∶1,年龄1~9岁,中位年龄4.2岁;IM组中,男53例,女40例,男女比例为1.32∶1,年龄1~12岁,中位年龄5.8岁。2组病例均以男性多见,前者好发年龄为幼儿期及学龄前期,后者好发年龄为学龄前期及学龄期。对2组临床表现、实验室检查及预后进行比较。结果 EB-HPS组在高热持续时间,肝脾肿大程度均明显高于IM组,差异有统计学意义(P<0.05);实验室检查示血细胞减少及血清铁蛋白升高,差异有统计学意义(P<0.05);肝功能损伤:EBV-DNA载量,纤维蛋白原减低,甘油三酯升高均明显高于IM组,差异有统计学意义(P<0.05)。EB-HPS组56.0%的患者可在骨髓检查中见噬血细胞现象,IM组中仅有9.0%的患者可在骨髓检查中见噬血细胞现象。EBV-HPS组死亡率为23.2%,而IM组为0.0%。结论儿童EBV-HPS好发年龄为幼儿期及学龄前期,IM好发于学龄前期及学龄期,对EBV感染后存在持续发热,肝脾淋巴结肿大患儿早期进行血常规、血清铁蛋白、肝功能、EBV-DNA载量、纤维蛋白原、甘油三酯及骨髓细胞检查能够及时对HPS进行诊断,减少误诊率及死亡率。时对HPS进行诊断,减少误诊率及死亡率。

Objective To compare the clinical manifestations and laboratory tests of EB virus hemophagocytic syndrome( EBV-HPS) and infectious mononucleosis( IM),to enhance the understanding of the EBV-HPS,reduce misdiagnosis and mortality. Methods Forty-five patients EBV-HPS and 93 patients with IM were analyzed retrospectively. In the EBV-HPS group,there were male 24 patients,female 21 patients,gender distribution 1. 14 ∶1,age range of one to nine,mean age 4. 2. In the IM group,there were male 53 patients,female 40 patients,gender distribution 1. 32∶1,age range of one to twelve,average age 5. 8. For both groups note a male dominance,the former high paroxysmal ages were infancy and pre-school age,and the latter high paroxysmal ages were pre-school age and school age. Results All the clinical manifestation,laboratory examination and prognosis were reviewed and analyzed in this study. The results showed that during the hyperpyrexia period,hepatosplenomegaly in the EBV-HPS group was apparently higher than that in the IM group,the results were statistically significant( P < 0. 05). Laboratory examination showed that hemocytopenia,serum ferritin rised,hepatic injury,EBV-DNA dose decrease,fibrinogen decrease,and triglyceride increase were apparently higher than those in the IM group. Almost 56. 0% of patients in the EBV-HPS group experienced the hemophagocytosis in bone marrow which were only developed 9. 0% of patients in the IM group. The regular antiviral treatment and symptomatic treatment of EBV-HPS were invalid with high fatality.The clinical course was especially dangerous. According to the HLH-2004 after solution treatment,the mortality of EBV-HPS patients was 23. 2%,while no death patients in the IM group. Conclusion EBV-HPS often presented in patients from infancy to pre-school age,and IM often present in patients from pre-school age to school age. The child who suffered from persistent fever and hepatosplenomegaly after EBV infection,should do the routine blood tests,serum ferrium,hepatic function test,EBV-DNA dose,fibrinogen,triglyceride,bone marrow cells to clarify HPS diagnosis. Only in this way can reduce the misdiagnosis rate and mortality.