The Prognostic Significance of Beta2 Microglobulin in Patients with Hemophagocytic Lymphohistiocytosis

[摘要] Objective. To determine the prognostic significance of beta2 microglobulin (?2-m) concentrations in patients with hemophagocytic lymphohistiocytosis (HLH), a rare disorder caused by pathologic activation of the immune system. Patients and Methods. The study population consisted of 74 patients diagnosed with HLH and 35 healthy controls. Serum ?2-m levels were measured using a latex agglutination photometric immunoassay. Results. Median serum ?2-m levels were significantly higher in HLH patients than in healthy controls (4.05 versus 1.5?mg/L; P<0.001) and were significantly higher in patients with lymphoma associated hemophagocytic syndrome (LAHS) than in patients with benign disease-associated HLH (4.2 versus 3.3?mg/L; P<0.001). Higher serum ?2-m levels were positively correlated with LAHS (P=0.005), abnormal lactate dehydrogenase concentrations (P=0.009), and hypoalbuminemia (P=0.003). ROC analysis showed that overall survival (OS) was significantly shorter in LAHS patients with serum ?2-m levels ?4.03?mg/L compared to <4.03?mg/L (P<0.001). Moreover, multivariate analysis showed that serum ?2-m level was an independent prognostic of OS (P=0.034) in patients with LAHS. Conclusion. High serum ?2-m levels and LAHS were associated with markedly poorer OS in patients with HLH. Serum ?2-m concentration was a powerful and independent prognostic factor for OS in patients with LAHS.

[学科分类] 生理学与病理学