摘要
目的:探讨噬血细胞综合征(hemophagocytic syndrome,HPS)的临床、实验室检查结果及预后影响因素,以提高对噬血细胞综合征的认识,了解影响预后的相关因素。方法:分析2012年01月至2020年01月我院收治的27例HPS患者的病因、临床表现、实验室检查结果及转归。结果:27例患者中,淋巴瘤相关HPS 10例,感染相关性HPS 9例,自身免疫病相关HPS 3例,1例伴UNC13D突变的原发性HPS,4例病因不明。HLH-2004诊断标准中各指标的符合率:发热、铁蛋白升高、NK细胞活性减低(100%),两系或三系血细胞减少(92.59%),sC D25升高(90.91%),脾大(85.19%),低纤维蛋白原血症(77.78%),高甘油三脂血症(74.07%),噬血现象(72.22%)。比较存活组和死亡组初诊时各实验室结果指标,血小板、纤维蛋白原、血清铁蛋白、乳酸脱氢酶水平两组差异有统计学意义(P<0.05)。多因素分析显示血小板减少是患者的独立预后危险因素(OR=0.955,95%CI:0.917~0.996,P=0.031)。结论:HPS临床表现多样,病情进展迅速,死亡率高,发热、至少两系血细胞减低、NK细胞活性降低、铁蛋白及sC D25水平明显升高在诊断中的灵敏度高。谷草转氨酶增高、β_(2)-微球蛋白增高、高密度胆固醇降低、低钠血症有利于诊断HPS。血小板计数减少是疾病的独立不良预后因素。
Objective:To explore the laboratory examination results of hemophagocytic syndrome(HPS)and its correlation analysis with prognosis,so as to improve the understanding of hemophagocytic syndrome and the relevant factors affecting prognosis.Methods:The etiology,clinical manifestations,laboratory results and outcomes of 27 patients with HPS from January 2012 to January 2020 were retrospectively analyzed.Results:Among the 27 patients,10 patients were malignant associated HPS,9 patients infection associated HPS,3 patients autoimmune disease associated HPS,1 patient primary HPS with UNC13D mutation,and 4 patients with unknown etiology.The coincidence rate of HLH-2004 diagnosis standard of each index included:Fever,elevated ferritin,NK cell activity decreased or absent(100%),2 or 3 cytopenia(92.59%),sCD25 increased(90.91%),splenomegaly(85.19%),fibrinogenemia(77.78%),hypertriglyceridemia(74.07%),hemophagocytes(72.22%).The differences in platelet,fibrinogen,serum ferritin and lactate dehydrogenase levels between the survival group and the death group were statistically significant(P<0.05).Multivariate analysis showed that thrombocytopenia was an independent prognostic risk factor(OR=0.955,95%CI:0.917~0.996,P=0.031).Conclusion:The clinical manifestations of HPS are diverse.The disease progresses rapidly.The mortality is high.Fever,at least two lines of blood cell hypoxia,NK cell activity decreased,ferritin and sCD25 levels significantly increased have high sensitivity in the diagnosis.Increased AST andβ_(2)-MG,reduced HDL cholesterol,and hyponatremia are beneficial to the diagnosis of HPS.Thrombocytopenia is an independent prognostic risk factor.
作者
李叶琼
冶秀鹏
王嘉漪
穆合塔尔·伊马木尼亚孜
马健
包慎
LI Yeqiong;YE Xiupeng;WANG Jiayi;Muhtar·Yimamniyaz;MA Jian;BAO Shen(People's Hospital of Ningxia Hui Autonomous Region,First Affiliated Hospital of Northwest University for Nationalities,Ningxia Yinchuan 750002,China)
出处
《现代肿瘤医学》
CAS
北大核心
2022年第4期689-692,共4页
Journal of Modern Oncology
基金
宁夏自然科学基金项目资助(编号:2018AAC03174)
宁夏人民医院振兴培育项目(编号:201920)。
关键词
噬血细胞综合征
发热
铁蛋白
NK细胞活性
脾大
预后因素
hemophagocytic syndrome
fever
ferritin
NK cell activity
splenomegaly
prognostic factors
