摘要
目的分析噬血细胞综合征(HPS)小儿患者的临床特点,以提高对本病早期诊断,减少误诊。方法分析11例HPS患儿的病因、临床症状、体征、实验室检查结果及转归。结果患儿以发热、肝脾肿大、淋巴结肿大为突出表现。其余表现为黄疸、皮疹和神经症状。实验室检查以肝功能损害、全血细胞减少、凝血功能障碍、骨髓有噬血现象为主。结论HPS可由多种病因所致,临床表现多样,病死率高,多以骨髓检查,以减少误诊和漏诊。
Objective To invrstigate the clinical features of hemophagocytic syndrome(HPS)and to improve its recognition,early diagnosis and reduce misdiagnosis.Methods A retrospective study was carried out to analyze the underlying diseases,clinical characteristics,laboratory findings and outcomes in 11 patients with HPS.Results HPS was clinically characterized by fever,hepatomegaly,splenomegaly,lymph adenopathy,and other features including jaundice,skin rash and nervous system involvement.Laboratory analysis indicated that liver dysfunction pancytomenia,coagulation abnormalities,and hemophagocytosis in bone marrow were the most prominent feature.Conclusion There are various underlying diseases,clinical manifestations and high mortality for HPS.Repeat bone marrow examinations are helpful for reducing misdiagnosis.
出处
《医学检验与临床》
2008年第2期25-26,共2页
Medical Laboratory Science and Clinics
关键词
噬血细胞综合征
小儿
家族性
EB病毒
Hemophagocytic syndrome
Child
Familial
Epstein-Barr virus
