摘要目的 比较传染性单核细胞增多症(infectious mononucleosis,IM)和EB病毒相关性噬血细胞综合征(EBV-associated hemophagocytic syndrome,EBV-AHS)的临床特点,分析IM患儿发生EBV-AHS的临床危险因素.方法 回顾性比较我院2000年1月至2006年4月430例IM和EBV-AHS患儿临床症状、体征和实验室检查特点,采用Logistic回归分析IM患儿发生EBV-AHS的临床危险因素.结果 (1)本组IM病例中EBV-AHS发生率为3.72%(16/430),EBV-AHS组患儿热程明显长于IM组患儿,体温峰值、肝脏和脾脏肿大程度均较IM组患儿明显,但咽峡炎发生率(37.5%)显著低于IM组(91.1%),差异均有统计学意义.(2)EBV-AHS组外周血三系均低于IM组,且变异淋巴细胞升高不明显,其比例(中位数10%)显著低于IM组(中位数18%),差异亦有统计学意义.(3)EBV-ASH组肝功能损害显著重于IM组,尤其乳酸脱氢酶(LDH)(中位数为2128.5 U/L)和天冬氨酸氨基转移酶(AST)(中位数为489 U/L)水平升高显著高于IM组,且常伴有高胆红素血症及低白蛋白血症.(4)多因素Logistic回归分析发现:热程＞10 d(OR=8.097)、LDH进行性升高＞1000 U/L(OR=7.998)、低白蛋白血症(OR=7.838)、中性粒细胞＜1.5×109/L(OR=7.587)和血小板＜100×109/L(OR=7.190)是本组IM患儿发生EBV-AHS的临床危险因素,本组EBV-AHS病死率高达50%.结论 绝大多数IM患儿呈良性自限性过程,约3.7%患儿进展为EBV-ASH.热程＞10 d、LDH＞1000U/L、低白蛋白血症、中性粒细胞＜1.5×109/L、血小板＜100 x 109/L是IM患儿发生EBV-AHS的临床危险因素,该病预后凶险,病死率高,多次骨髓检查有助于及时诊断.

abstractsObjective To compare the clinical features of infectious mononucleosis(IM)and Epstin-Barr virus(EBV)-associated hemophagocytic lymphohistiocytosis(EBV-AHS)and identify the clinical risk factors in IM patients cornplicated with EBV-AHS. Method A retrospective study was carried out to analyze the clinical and laboratory data of 414 IM and 16 EBV-AHS children from January.2000 to April.2006.Then Logistic regression was used to identify the risk factors for progression to EBV-ASH.Resuits (1) The incidence of EBV-AHS among the IM children was 3.72%(16/430).There were significant differences between EBV-ASH and IM children in duration of fever(20 days vs.7 days,P＜0.001),the peaks of fever(40.0℃ vs.39.0 ℃,P＜0.001),the degree of hepatomegaly(3.5 cm vs 2.0cm below costal arch.P＜0.05)and splenomegaly(2.75 cm vs.1.0 cm below costal arch,P＜0.05),while the incidence of isthmitis in EBV-AHS patients was markedly lower than that of IM patients(37.5%vs.91.1%.P＜0.01).(2)Pancytopenia was often observed in EBV-AHS patients and significant differences between two groups were found in median of leukocytes(3.1×109/L vs.12.8×109/L,P＜0.001),median of neutrophils(0.53×109/L vs.3.17 x 109/L,P＜0.001),mean of hemoglobin(80g/L vs.120 g/L.P＜0.001)and median of platelet(27.5×109/L vs.183×109/L,P＜0.001).(3)Hepatic derangement evidenced by elevated serum enzymes,hyperbilirubinemia and hypoalbuminemia in EBV-ASH children was much more severe than that in IM children.especially LDH level(2128.5 U/L vs.445 U/L,P＜0.001)and AST level(489 U/L vs.59 U/L,P＜0.001).(4)The clinical risk factors for IM patients progressing to EBV-ASH were lasting fever ≥ 10 days ( OR = 8. 097, P = 0. 008 ), LDH ＞ 1000U/L ( OR = 7. 998, P = 0. 033 ), hypo-albuminemia ( albumine ＜ 35g/L, OR = 7. 838, P = 0. 038 ),neutrophils ＜ 1.5 x 109/L ( OR = 7. 587, P = 0. 022) and Plt＜ 100 x 109/L ( OR = 7. 190, P = 0. 027 ).The mortality of EBV-AHS in the patients was 50. 0% (8/16). Conclusion Most of IM children clinically manifest self-limited process, but about 3.72% of whom may progress to fatal EBV-ASH. The clinical risk factors for EBV-AHS are lasting fever ＞ 10 days, LDH ＞ 1000 U/L, hypoalbuminemia, neutropenia and Plt ＜ 100 x 109/L. EBV-ASH is an extremely dangerous state with high mortality. Repeated bone marrow examinations are helpful for diagnosis in time.