以急性肝功能衰竭为首发表现的噬血细胞综合征11例临床分析

Clinical features of eleven cases with hemophagocytic syndrome presenting as acute liver failure

目的通过对11例以急性肝功能衰竭为首发症状的噬血细胞综合征（hemophagocyticsyndrome，HPS）患儿的临床特征进行分析，提高对本病的早期识别。方法收集中国医科大学附属盛京医院2011年9月至2015年2月收治的以急性肝功能衰竭为首发表现的HPS患儿11例，对其临床表现、实验室检查结果、治疗方法、预后进行综合分析。结果HPS可以急性肝功能衰竭为首发症状，其特点为凝血功能严重异常，ALT、AST升高，纤维蛋白原降低。11例患儿均有大于1周的热程和脾脏肿大；治疗上在应用肾上腺皮质激素及静注丙种球蛋白等抑制单核巨噬细胞系统的激活，早期依托泊苷进行化疗等基础上积极予以保肝降酶等综合治疗，部分病例伴有严重出血倾向，予血浆置换及连续血液透析滤过治疗。11例患儿中，存活1例，4例因多脏器功能衰竭死亡，自动出院6例随访均死亡。结论不明原因急性肝功能衰竭并伴有发热和血细胞减少的患儿应警惕HPS，进行早期诊断和治疗以降低病死率。

Objective To investigate the clinical features of eleven cases of acute liver failure as the initial presentation of hemophagocytic syndrome （ HPS ）, in order to improve the early diagnosis. Methods Eleven cases of acute liver failure as the initial presentation of HPS admitted in PICU of Shengjing Hospital affiliated to China Medical University from September 2011 to February 2015 were investigated, the clinical manifestations,laboratory findings,therapy methods and prognosis were analyzed. Results Eleven cases of HPS had the initial symptom of acute liver failure accompanied by severe coagulation abnormalities,increase of alanine aminotransferase and aspartate aminotransferase, decrease of fibrinogen. All 11 cases with speno- megaly had more than 1 week thermal process. Glucocorticoid and gamma globulin were used to inhibit the activation of monocyte-macrophage cell system. Chemotherapy such as Etoposide were used as the basic treat- ment in the early stage. Plasma exchange and continuous hemodialysis and filtration were used in severe cases with bleeding tendency. One of these 11 children survived, 4 cases died of multiple organ dysfunction syndrome, and discharged six cases were followed up for mortality. Conclusion Unexplained acute liver failure, fever and cytopenias may suggest HPS ,the mortality rate can be reduced by early diagnosis and treatment.