婴幼儿噬血细胞性淋巴组织细胞增生症115例临床分析

发布时间：2018-10-14 14:12

【摘要】：目的:分析婴幼儿噬血细胞性淋巴组织细胞增生症(hemophagocytic lymphohistocytosis,HLH)患儿的病历资料,掌握该病的临床特征规律。方法:回顾性分析2011年9月至2016年9月在我院诊治的115例3岁以下HLH患儿的临床资料,并按年龄分为新生儿期(1月)、婴儿期(1月-1岁)、幼儿期(1岁-3岁)三组,按病因分为EBV感染组和非EBV感染组,按治疗方案分为HLH-2004治疗方案组和非HLH-2004治疗方案组,探讨患儿的预后因素、以及治疗方案的选择。结果:本组患者总数115例,其中男性患儿66例(57.39%),女性患儿49例(42.61%),男女之比为1.35:1。新生儿(1月)14例(12.17%),婴儿(1月-1岁)34例(29.57%),幼儿(1岁-3岁)67例(58.26%),幼儿期为该年龄段患儿的发病高峰期。感染引起HLH的患儿共66例,是最常见的病因,其中EBV感染49例;肿瘤相关性5例;原发性9例;风湿免疫相关性1例;原因不明性34例。临床表现主要为发热、脾大、呼吸道症状、肝大,此外可见皮肤黏膜苍白、浆膜腔积液、淋巴结肿大、水肿、黄染、出血症状、皮疹,少数可合并肾功能不全、循环系统症状、中枢神经系统症状。实验室结果主要有两系或三系减低(108例)、血清铁蛋白升高(106例)、谷草转氨酶升高(106例)、谷丙转氨酶升高(97例)、乳酸脱氢酶升高(93例)、纤维蛋白原减低(92例)。除此之外,电解质失衡、骨髓噬血现象、血清白蛋白含量减少、总胆红素值增加、甘油三酯增多等也较常见。通过对早期缓解组与未缓解组患者的相关实验室及临床指标进行比较,两组在初诊时WBC、PLT、APTT、PT、TG、DBIL有明显差别,具有统计学意义。比较HLH-2004治疗方案与非HLH-2004方案治疗HLH的早期缓解情况的差异具有统计学意义。比较EB病毒感染组患者采用HLH-2004方案与非HLH-2004方案治疗HLH的早期缓解情况的差异具有统计学意义。结论:1.感染是婴幼儿继发性HLH的常见病因,其中EB病毒感染最常见;2.婴幼儿HLH患儿早期的WBC、PLT计数减少及APTT、PT、TG、DBIL明显升高可能与预后不良有关;3.婴幼儿HLH患儿确诊后及时应用HLH-2004方案可提高早期缓解率。
[Abstract]:Objective: to analyze the clinical characteristics of hemophagocytic lymphohistiocytosis (hemophagocytic lymphohistocytosis,HLH) in infants. Methods: the clinical data of 115 children with HLH under 3 years old who were diagnosed and treated in our hospital from September 2011 to September 2016 were analyzed retrospectively and divided into three groups according to their age: neonatal stage (1 month), infancy (1 month-1 year old), infant stage (1 year-3 years old). According to the etiology, EBV infection group and non-EBV infection group were divided into HLH-2004 treatment group and non-HLH-2004 treatment group. The prognostic factors and the choice of treatment plan were discussed. Results: there were 115 cases in this group, 66 cases were male (57.39%), 49 cases were female (42.61%), the ratio of male and female was 1.35: 1. 14 newborns (12.17%), 34 (29.57%) infants (1-1 years old) and 67 (58.26%) infants (1-3 years old). The most common cause of HLH was EBV infection in 49 cases, tumor-related in 5 cases, primary in 9 cases, rheumatic immunity in 1 case and unknown cause in 34 cases. The main clinical manifestations were fever, splenomegaly, respiratory symptoms, hepatomegaly, skin and mucosal paleness, serous cavity effusion, lymphadenopathy, edema, yellow stain, bleeding symptoms, rash, a few may be associated with renal insufficiency, circulatory system symptoms, Central nervous system symptoms The results were as follows: decrease of two or three lines (108 cases), increase of serum ferritin (106 cases), increase of alanine aminotransferase (106 cases), increase of alanine aminotransferase (97 cases), increase of lactate dehydrogenase (93 cases) and decrease of fibrinogen (92 cases). In addition, electrolyte imbalance, bone marrow hemophagocytosis, serum albumin content, total bilirubin increase, triglyceride increase are also more common. By comparing the laboratory and clinical indexes between the early remission group and the non-remission group, there was a significant difference in WBC,PLT,APTT,PT,TG,DBIL between the two groups at the first visit. The difference between HLH-2004 regimen and non-HLH-2004 regimen in early remission of HLH was statistically significant. There were significant differences in early remission of HLH between HLH-2004 regimen and non-HLH-2004 regimen in patients with EB virus infection. Conclusion: 1. Infection is a common cause of infantile secondary HLH, in which EB virus infection is the most common; 2. The decrease of WBC,PLT count and the obvious increase of APTT,PT,TG,DBIL in infants with HLH may be related to poor prognosis. 3. The early remission rate of infants with HLH can be improved by using HLH-2004 regimen in time after diagnosis.
【学位授予单位】：郑州大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R725.5

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