肝功能异常为主要临床表现的噬血细胞综合征10例临床分析

The clinical analysis of hepatic dysfunction caused by hemophagocytic syndrome of 10 cases

目的通过对10例以肝功能异常为主要临床表现的噬血细胞综合征病例进行分析,探讨本病的临床特点,提高对本病的认识。方法对10例患者的病史、临床表现和实验室检查结果进行综合分析。结果噬血细胞综合征(HPS)可表现为严重的肝功能损害,其特点为胆红素明显升高,以直接胆红素升高为主,以及ALT、AST及GGT升高。结论不明原因肝功能异常并伴有发热和血细胞减少的患者应警惕HPS,早期诊断和治疗。

Objective To investigate the clinical features of hepatic dysfunction caused by hemophagocytic syndrome（HPS）.Methods Medical records,clinical manifestations and laboratory findings were analyzed.Results Characteristics of hepatic dysfunction caused by hemophagocytic syndrome were increase of direct bilirubin level,and increase of alanine aminotransferase（ALT）,aspartate aminotransferase（AST） and γ-glutamyltransferase（GGT）.Conclusion Awareness of diagnosis of HPS is crucial when patients have unknown hepatic dysfunction,fever,hepatosplenomegaly and pancytopenia.