本文選題：噬血細(xì)胞綜合征 + 診斷��； 參考：《山西醫(yī)科大學(xué)》2017年碩士論文

【摘要】：目的:總結(jié)24例噬血細(xì)胞綜合(Hemophagocytic syndrome,HPS)患者的臨床及實(shí)驗(yàn)室特征,并分析其對(duì)預(yù)后的影響,同時(shí)提高對(duì)該疾病的認(rèn)識(shí)。方法:收集2012年10月至2016年10月山西醫(yī)科大學(xué)第二醫(yī)院診治的24例HPS患者的資料,包括:臨床特點(diǎn)、實(shí)驗(yàn)室檢查、治療方案及轉(zhuǎn)歸,并進(jìn)行回顧性分析。24例患者根據(jù)初次治療結(jié)局分為生存組與死亡組。應(yīng)用SPSS17.0軟件對(duì)數(shù)據(jù)進(jìn)行分析,計(jì)量資料以均數(shù)±標(biāo)準(zhǔn)差表示,采用兩獨(dú)立樣本的t檢驗(yàn),計(jì)數(shù)資料采用卡方檢驗(yàn)(Fisher確切概率法),P0.05為差異有統(tǒng)計(jì)學(xué)意義。結(jié)果:1.24例確診的HPS患者中,男性41.67%,女性58.33%,男:女=0.71:1。發(fā)病年齡范圍15-78歲之間,中位年齡為39.5歲。2.24例患者均為繼發(fā)性HPS,13例(54.17%)患者繼發(fā)于感染,其中EB病毒感染10例(41.67%),巨細(xì)胞病毒感染2例(8.33%),EB病毒合并巨細(xì)胞病毒感染1例(4.17%)。淋巴瘤相關(guān)者4例(16.67%),7例(29.17%)患者病因未明確。3.臨床表現(xiàn)包括:發(fā)熱(95.83%),脾大(83.33%),淺表淋巴結(jié)腫大(66.67%),體重下降(54.17%),消化系統(tǒng)癥狀(75%),呼吸系統(tǒng)癥狀(45.83%),出血(25%),神經(jīng)系統(tǒng)癥狀(45.83%),漿膜腔積液(33.33%),皮疹(8.33%)。4.實(shí)驗(yàn)室檢查結(jié)果:21例(87.5%)患者存在兩系或三系血細(xì)胞減少,此外以下指標(biāo)也可見明顯下降或減少:FIB(87.5%)、白蛋白(87.5%)、NK細(xì)胞比例(66.67%)。而以下指標(biāo)則明顯升高: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%)患者出現(xiàn)骨髓噬血細(xì)胞現(xiàn)象。另外,10例(41.67%)患者發(fā)現(xiàn)多腫瘤標(biāo)志物的異常。5.10例患者采用HLH-2004方案(地塞米松+VP16+環(huán)孢素),3例患者采用VP16+甲強(qiáng)龍方案,1例患者單用VP16,2例患者單用激素治療,1例患者采用E-CHOP方案,1例患者采用COEP方案,6例患者予對(duì)癥治療(抗感染、保肝、調(diào)節(jié)免疫等)。經(jīng)治療后,12例因疾病進(jìn)展死亡,12例病情好轉(zhuǎn)后出院。6.存活組與死亡組確診時(shí)的的NK細(xì)胞比例(P=0.04)、LDH(P=0.032)、SF(P=0.023)水平差異有統(tǒng)計(jì)學(xué)意義。結(jié)論:HPS病因復(fù)雜,繼發(fā)性HPS以感染和腫瘤最多見。臨床表現(xiàn)以發(fā)熱、脾大、消化系統(tǒng)癥狀最常見。實(shí)驗(yàn)室檢查可見血細(xì)胞減少、NK細(xì)胞比例下降、SF升高、LDH升高、骨髓可見噬血細(xì)胞及肝功能指標(biāo)異常,部分患者還可見多腫瘤標(biāo)志物異常。目前主要的治療方案為包含VP16的化療方案。發(fā)病初SF過高、NK細(xì)胞比例降低及LDH升高是影響預(yù)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 鹵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.
【學(xué)位授予單位】：山西醫(yī)科大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2017
【分類號(hào)】：R55

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