噬血细胞综合征临床特点及预后影响因素分析(附27例)

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...

Analysis of clinical features and prognostic factors of hemophagocytic syndrome(27 cases attached)

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 andβ₂-MG,reduced HDL cholesterol,and hyponatremia are beneficial to the diagnosis of HPS.Thrombocytopenia is an independent prognostic risk factor.