实验室诊断指标对嗜血细胞综合征的诊断价值

The diagnostic value of laboratory features for hemophagocytic syndrome.

目的探讨实验室诊断指标对嗜血细胞综合征(HPS)的诊断价值。方法对56例HPS患者的病因及实验室诊断结果进行回顾性分析。结果HPS患者常以发热,肝、脾、淋巴结肿大,血细胞减少为主要表现,伴有不同程度的肝功能和凝血异常,骨髓组织细胞增多,可见到噬血组织细胞。血清铁蛋白(SF)、三酰甘油(TG)及乳酸脱氢酶(LDH)升高;纤维蛋白原(FIB)降低。结论外周血细胞减少、骨髓涂片中噬血组织细胞≥1%、高TG、高SF、高LDH及低FIB都是诊断HPS的重要实验室指标,因此提高了对HPS实验室特征认识,有利于加强HPS诊断和有效治疗。

Objective To explore the diagnostic value of laboratory examinations for hemophagocytic syndrome（HPS）.Methods The causes of disease and laboratory results in 56 patients with hemophagocytic syndrome were respectively reviewed.Results The main manifestations of HPS were fever,hepatosplenomegaly,lymphadenectasis,and cytopenia,accompanied by different degrees of hepatic and coagulative dysfunction.The number of histiocytes was increased and hemophagocytic macrophages were found in bone marrow.Laboratory findings included elevated serum ferritin,triglycerides,and lactate dehydrogenase,and decreased fibrinogen.Conclusion Cytopenia,hemophagocytic cells in bone marrow ≥1%,elevated serum triglycerides,ferritin and lactate dehydrogenase,and low fibrinogen level are important experimental indexes for diagnosing hemophagocytic syndrome.This study indicates the value of laboratory findings in the diagnosis and treatment of patients with HPS.