ABSTRACT
<p><b>OBJECTIVE</b>To study the prognostic significance of coagulation disorders in children with hemophagocytic syndrome (HPS).</p><p><b>METHODS</b>Thirty-five children with HPS were retrospectively studied to analyze the etiology, clinical characteristics, laboratory results and treatment outcomes.</p><p><b>RESULTS</b>After treatment, 27 of the 35 HPS patients survived, and the other 8 cases died. All cases were treated according to the HLH-2004 protocol, but etoposide (VP-16) was not used in 10 of them. The response rate in patients who received VP-16 (22/25, 88%) was significantly higher than that in those not receiving VP-16 (5/10, 50%) (P<0.05). Compared with the survival group, the dead group had significantly lower platelet count, fibrinogen level, and VP-16 utilization rate (P<0.05) but significantly longer activated partial thromboplastin time and prothrombin time (P<0.05).</p><p><b>CONCLUSIONS</b>Coagulation function can be used as an indicator of disease outcome. It is essential for improving the clinical outcome of HPS to monitor the coagulation function during treatment, detect and correct abnormalities in time, and provide treatment strictly according to the HLH-2004 protocol.</p>