获得性噬血细胞性淋巴组织细胞增多症合并肝功能损害的初步分析

本研究探讨获得性噬血细胞性淋巴组织细胞增多症（hemophagocytic lymphohistiocytosis，HLH）肝功能损害的临床特点。对18例获得性HLH合并肝功能损害患者的临床资料进行初步分析，了解肝功能损害的特点，以及肝功能损害与获得性HLH病因和预后之间的关系。结果表明：获得性HLH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、低蛋白血症和黄疸。非肿瘤继发HLH组患者门冬氨酸转氨酶（AST）和直接胆红素（DBil）水平均明显高于肿瘤继发组HLH患者（P〈0．05）。门冬氨酸转氨酶（AST）和乳酸脱氢酶（LDH）增高提示预后不良（P〈0．05）。结论：肝功能损害是获得性HLH常见的器官功能障碍，其肝功能损害程度可能与获得性HLH病因和预后相关。

Preliminary Clinical Analysis of the Hepatic Dysfunction in Patients with Acquired Hemophagocytic Lymphohistiocytosis

The aim of this study was to investigate the clinical features of acquired hemophagocytic lymphohistiocytosis （HLH） complicated with hepatic dysfunction. 18 cases of acquired HLH were analyzed. The characteristics of hepatic dysfunction, the relationship between hepatic dysfunction and the cause, as well as prognosis of the acquired HLH were preliminarily analysed. The results indicated that characteristics of hepatic dysfunction in acquired HLH patients were hypoproteinemia, jaundice and increase of L-asparate aminotransferase （AST） and lactate dehydrogenase （LDH） levels. The legel of AST and direct bilirubin （DBil） in the non-malignancy associated hemophagocytic lymphohistiocytosis group were higher than that in malignancy-associated hemophagocytic lymphohistiocytosis group（p 〈 0.05 ）. And the increase of LDH and AST levels indicated poor prognosis（p 〈 0.05）. In conclusion, liver damage is a common organ functional disorder in patients with acquired HLH, which may be correlated to the cause and the prognosis of acquired HLH.