噬血细胞现象在诊断噬血细胞性淋巴组织细胞增多症中意义的探讨

Significance of Hemophagocytosis in Diagnosis of Hemophagocytic Lymphohistiocytosis

本研究探讨噬血细胞现象在诊断噬血细胞性淋巴组织细胞增多症(hemophagocytic lymphohistiocytosis,HLH)中的意义。收集2005年6月至2008年10月继发性HLH疑似病例61例,根据HLH-2004诊断标准分为确诊组及排除组,比较各组骨髓、脾或淋巴结活检中噬血细胞现象的发生率。结果表明,61例疑似患者中有43例确诊为继发性HLH,18例排除HLH;确诊组中有33例发生噬血细胞现象,排除HLH组中有4例出现噬血细胞现象;噬血细胞现象在诊断HLH时的敏感性为76.7%,特异性为77.8%。结论:噬血细胞现象在诊断大部分继发性HLH的中具有重要的意义,但没有噬血细胞现象并不能排除HLH的诊断。

The study was aimed to explore the diagnostic significance of hemophagocytosis in the patients with hemophagocytic lymphohistiocytosis（HLH）. 61 suspected HLH patients from June 2005 to October 2008 were enrolled in the study. The suspected HLH patients were divided into confirmed group （43 out of 61 ） and excluded group （ 18 out of 61 ） according to HLH-2004 diagnostic criteria. The incidences of hemophagocytosis in bone marrow, spleen or lymph nodes were compared in all groups. The results indicated that the hemophagocytosis in bone marrow, spleen or lymph nodes was found in 33 patients of confimed group, while the hemophagocytosis was found in 4 patients of excluded group. The sensitivity of hemophagocytosis for the diagnosis of HLH was 76.7%, and its specificity was 77.8%. In conclusion, hemophagocytosis is a helpful marker for the diagnosis of most but not all HLH patients, however, the lack of hemophagocytosis does not mean to exclude the diagnosis of HLH.