目的：分析儿童噬血细胞性淋巴组织增生综合征（HLH）实验室诊断指标的改变及其原因，帮助临床快速准确的诊断。方法回顾性分析2011年1月至2013年3月该院收治的13例H L H患者的实验室诊断资料。结果 H L H首发症状常为发热，肝、脾及淋巴结肿大，继而全血细胞减少，肝功能及凝血功能不同程度异常，骨髓组织细胞增多，可见噬血组织细胞。外周血细胞二系减少者占23．1％，三系减少者占69．2％，三酰甘油（T G ）升高者占69．2％，血清铁蛋白（S F ）升高者占100．0％，乳酸脱氢酶（LDH）升高者占100．0％，丙氨酸氨基转移酶（ALT）升高者占69．2％，纤维蛋白原（FIB）下降者占76．9％。结论对不明原因持续高热，肝、脾及淋巴结肿大，外周血细胞二系或三系降低患儿均应进行HLH的一系列相关检查如肝功能、血脂、血凝、细胞免疫学及骨髓细胞形态学等初步诊断和判断预后，有条件者应进一步进行分子生物学检测以确诊。%Objective To summarize the cause and the level change of laboratory indicators in children with hemphagocytic lym‐phohistiocytosis(HLH) .Methods Retrospectively analyze the cause and the diagnose features of the 13 HLH patients .Results The first symptom of the desease was hyperpyrexia as well as the hepatosplenomegaly and the lymphadenectasis .Then it went with the peripheral cytopenia in two or three lineages ,different degrees of the liver dysfunction and the coagulation abnormalities .The number of histiocytes increased and hemophagocytic macrophages were found in bone marrow .It also showed that the elevated ser‐um triglycerides ,serum ferritin ,lactate dehydrogenase and glutamic‐pyruvic transaminase and decreased fibrinogen in laboratory findings .Conclusion Cytopenia(in two or three lineages ,especially the platelet) ,the elevated serum triglycerides ,serum ferritin , lactate dehydrogenase ,glutamic‐pyruvic transaminase ,decreased fibrinogen and the hemophagocytic macrophages in bone marrow (≥1% ) are important indicators in hemphagocytic lymphohistiocytosis diagnosis ,which could help us know the change of the HLH laboratory features better and diagnose the disease correctly .
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