Hội chứng thực bào máu: tổn thương gan và kết quả điều trị trong giai đoạn đầu tại bệnh viện Nhi Đồng I từ tháng 1/2010 -8/2011

Study objective: to determine the association between liver damage and response to the initial treatment (after 8 weeks) of Hemaphagocytic Iymphohistiocytosis. Methods: A case. series study was conducted in 51 HLH patients age 16 years, who were hospitalized at The Children Hospital No.1, HCM city from Jan 2010 to August 2011. All patients were treated with the HLH 2004 guideline for the initial period, and classified into two groups: liver damage and non. - liver damage. The outcome of patients also has two groups: response to treatment, non - reponse to treatment. Clinical, laboratory data at the first day of diagnosis and outcome of HLH patients were analyzed to find factors associated liver damage group and non - liver damage group. Results: 51 patients were enrolled. The rate of liver damage was 25.4 percent. In the liver damage group, the rate of patients having the number of white blood cell below 5000/mm3, of neutrophile below 500/mm3 are higher those in the non - liver damage group (92.8 percent and 64.9 percent; 57.1 percent and 29.7 percent, respectively). The rate of triglyceridemia 7 mmol/L and ferritinemia 5000 ug/L also higher. After the initial treatment, the rate of reponse to treatment in the liver damage group and non - liver damage group is 5/14 and 10/37 in respectively. Conclusion: Patients in liver damage group has high ferritinemia, triglyceridemia but low white blood cell and neutrophil.