成人获得性噬血细胞综合症肝功能损害临床资料分析

目的探讨成人获得性噬血细胞综合症（hemophagocyticsyndrome，HPS）肝功能损害的临床特点。方法回顾性分析郑州大学附属肿瘤医院2010年3月-2012年8月收治的24例成人获得性嗜血细胞综合症合并肝功能损害的患者，了解肝功能损害的特点，以及肝功能损害与获得性HPS病因和预后之间的关系。结果获得性HPS患者肝功能损害的特点为乳酸脱氢酶（LDH）及门冬氨酸转氨酶（AST）升高、低蛋白血症、高脂血症和黄疸。肿瘤继发HPS组乳酸脱氢酶（LDH）水平明显高于非肿瘤继发HPS组（P〈0．05）。门冬氨酸转氨酶（AST）增高、白蛋白（ALB）降低提示预后不良（P〈0．05）。24例患者中12周内共死亡15例，占62．50％。结论肝功能损害是获得性HPS常见的器官功能损害，其肝功能损害程度可能与获得性HPS的病因和预后相关。

Clinical analysis of adult acquired hemophagocytic syndrome patients with hepatic dysfunction 24 Cases

Objective To investigate the clinical features of acquired hemophagocytic syndrome（HPS） complicated with hepatic dysfunction. Methods Retrospective analysis of 24 cases of adult hemophagocytic syndrome with hepatic dys- function who received treatment in our hospital between March. 2010 and Aug. 2012. The characteristics of hepatic dys- function, the relationship between hepatic dysfunction and the cause, as well as prognosis of the acquired HPS were pre- lim inarily analysed. Results The characteristics of hepatic dysfunction in acquired HPS patients were hypoproteine- mia, hyperlipidemia, jaundice and increase of aspartate aminotransferase （AST） and lactate dehydrogenase （LDH） lev- els. The level of LDH in the malignancy associated hemophagocytic lymphohistioeytosis group were higher than that in non - malignancy associated hemophagocytic lymphohistiocytosis group （ P 〈 0. 05 ）. And the increase of AST levels or the reduce of albumin （ALB） indicated poor prognosis （ P 〈 0.05 ）. Conclusions Liver damage is a common organ functional disorder in patients with acquired HPS , which may be correlated to the cause and the prognosis of acquired HPS.