噬血细胞综合征24例临床分析

发布时间：2018-04-23 08:19

本文选题：噬血细胞综合征 + 诊断　；参考：《山西医科大学》2017年硕士论文

【摘要】：目的:总结24例噬血细胞综合(Hemophagocytic syndrome,HPS)患者的临床及实验室特征,并分析其对预后的影响,同时提高对该疾病的认识。方法:收集2012年10月至2016年10月山西医科大学第二医院诊治的24例HPS患者的资料,包括:临床特点、实验室检查、治疗方案及转归,并进行回顾性分析。24例患者根据初次治疗结局分为生存组与死亡组。应用SPSS17.0软件对数据进行分析,计量资料以均数±标准差表示,采用两独立样本的t检验,计数资料采用卡方检验(Fisher确切概率法),P0.05为差异有统计学意义。结果:1.24例确诊的HPS患者中,男性41.67%,女性58.33%,男:女=0.71:1。发病年龄范围15-78岁之间,中位年龄为39.5岁。2.24例患者均为继发性HPS,13例(54.17%)患者继发于感染,其中EB病毒感染10例(41.67%),巨细胞病毒感染2例(8.33%),EB病毒合并巨细胞病毒感染1例(4.17%)。淋巴瘤相关者4例(16.67%),7例(29.17%)患者病因未明确。3.临床表现包括:发热(95.83%),脾大(83.33%),浅表淋巴结肿大(66.67%),体重下降(54.17%),消化系统症状(75%),呼吸系统症状(45.83%),出血(25%),神经系统症状(45.83%),浆膜腔积液(33.33%),皮疹(8.33%)。4.实验室检查结果:21例(87.5%)患者存在两系或三系血细胞减少,此外以下指标也可见明显下降或减少:FIB(87.5%)、白蛋白(87.5%)、NK细胞比例(66.67%)。而以下指标则明显升高:TG(58.33%)、ALT(83.33%)、AST(75%)、TBIL(62.5%)、DBIL(62.5%),IBIL(37.5%)、LDH(79.17%)及SF(95.83%)。20例(83.33%)患者出现骨髓噬血细胞现象。另外,10例(41.67%)患者发现多肿瘤标志物的异常。5.10例患者采用HLH-2004方案(地塞米松+VP16+环孢素),3例患者采用VP16+甲强龙方案,1例患者单用VP16,2例患者单用激素治疗,1例患者采用E-CHOP方案,1例患者采用COEP方案,6例患者予对症治疗(抗感染、保肝、调节免疫等)。经治疗后,12例因疾病进展死亡,12例病情好转后出院。6.存活组与死亡组确诊时的的NK细胞比例(P=0.04)、LDH(P=0.032)、SF(P=0.023)水平差异有统计学意义。结论:HPS病因复杂,继发性HPS以感染和肿瘤最多见。临床表现以发热、脾大、消化系统症状最常见。实验室检查可见血细胞减少、NK细胞比例下降、SF升高、LDH升高、骨髓可见噬血细胞及肝功能指标异常,部分患者还可见多肿瘤标志物异常。目前主要的治疗方案为包含VP16的化疗方案。发病初SF过高、NK细胞比例降低及LDH升高是影响预后的不良因素。
[Abstract]:Objective: to summarize the clinical and laboratory features of 24 patients with hemophagocytic syndromes (HPSs), and to analyze its influence on prognosis and to improve the understanding of the disease. Methods: from October 2012 to October 2016, 24 patients with HPS were collected from the second Hospital of Shanxi Medical University, including clinical characteristics, laboratory examination, treatment scheme and outcome. According to the outcome of primary treatment, 24 patients were divided into survival group and death group. SPSS17.0 software was used to analyze the data, the measurement data were expressed as mean 卤standard deviation, the t test of two independent samples was used, and the counting data were statistically significant by using chi-square test and Fisher exact probability method (P0.05). Results among the 1. 24 confirmed HPS patients, male was 41. 67 and female was 58. 33. Male: female was 0. 71: 1. The onset age ranged from 15 to 78 years old. The median age was 39.5 years. The median age was 39.5 years. The median age was 39.5 years. The median age was 39.5 years. The median age was 39.5 years. All the patients were secondary infection. Among them, 10 cases were infected with Epstein-Barr virus (EBV), 10 cases were infected with Epstein-Barr virus (EBV), and 2 cases were infected with CMV. 2 cases were infected with Epstein-Barr virus (EBV) and cytomegalovirus (CMV) in 1 case. The etiology of 4 cases with lymphoma associated with 16. 67% and 7 cases with 9. 17%) is not clear. 3. 3. The clinical manifestations included: fever 95.83, splenomegaly 83.33, superficial lymphadenopathy 66.67, weight loss 54.17, digestive system symptoms 75, respiratory symptoms 45.83, hemorrhage 255.833, serous cavity accumulations 33.3333, rash 8.33%. The results of laboratory examination were as follows: there were two or three lines of blood cell decrease in 21 patients. In addition, the percentage of NK cells in 87.5% FIBN was significantly decreased or decreased in the following indexes. The percentage of NK cells in 87.5% Alb was 66.67%. However, the following indexes were obviously increased: TGG 58.33 and alt 83.33 and AST 75, TBI 62.5 and DBIL62.5) and SF(95.83%).20 (79.17) and SF(95.83%).20 (83.33) patients had hemophagocytosis in bone marrow. 5. 10 patients were treated with HLH-2004 regimen (dexamethasone VP16 cyclosporine 3 patients with VP16 methylenolone regimen 1 patient with VP16 methylenolone alone 2 patients with single hormone therapy and 1 patient with steroid therapy) and 10 patients with multiple tumor markers were found to be abnormal with multiple tumor markers. 5. 10 patients were treated with HLH-2004 regimen (3 patients with dexamethasone VP16 cyclosporine) and 1 patient with VP16 methylenolone alone. One patient with E-CHOP regimen was treated with COEP regimen. 6 patients were treated with symptomatic therapy (anti-infection). Liver protection, immune regulation, etc. After treatment, 12 patients died as a result of disease progression, 12 patients recovered and discharged. There was significant difference in the proportion of NK cells between the survival group and the death group at the time of diagnosis. Conclusion the etiology of HPS is complex, and infection and tumor are the most common secondary HPS. Fever, splenomegaly and digestive system symptoms are the most common clinical manifestations. Laboratory examination showed that the proportion of blood cells decreased and NK cells decreased, SF increased and LDH increased, blood macrophages and liver function were abnormal in bone marrow, and many tumor markers were abnormal in some patients. At present, the main treatment regimen is chemotherapy including VP16. The low proportion of NK cells and the increase of LDH were the bad prognostic factors.
【学位授予单位】：山西医科大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R55

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