An Analysis Of 31 Cases With Hemophagocytic Syndrome In Children

Objective: Through the retrospective analysis of clinical manifestation,laboratory examination and treatment of 31 children with hemophagocytic syndrome diagnosed and treated in pediatric department of our hospital,We aim to improve the overall understanding of hemophagocytic syndrome in children so as to achieve the purpose of early diagnosis,early treatment and improvement of prognosis.Method: A retrospective study was performed on 31 pediatric patients with HLH who were admitted to Second Hospital of He bei Medical Univercity from January 2014 to December 2018.Results:1.Among the 31 patients,the onset age was 4 months to 13 years and11 months,and the median age was 1years and 9months.Infectious HLH is the most common cause.The clinical manifestations were different among them.Most of them had persistent fever,hepatosplenomegaly,cytopenia,ferritin elevation,abnormal liver function and bone marrow hemophagy.2.HLH-94 or HLH-2004 regimen was mainly used in the treatment.There were 4 cases with hemophagocytic syndrome treated with dexamethasone alone,2 cases with dexamethasone and cyclosporine,12 cases with dexamethasone and etoposide,7 cases with dexamethasone and etoposide cyclosporine and 6 cases with other methods.Other methods include rescue therapy,allogeneic hematopoietic stem cell transplantation and blood purification.After treatment,17 cases achieved clinical remission,2 cases were clinically effective,3 cases abandoned or transferred to other hospital,and 9 cases died.Conclusion:1.The clinical manifestation of hemophagocytic syndrome is not specific,its progress is rapid and early diagnosis is difficult.Finally,the mortality of paitents is higher.2.Once hemophagocytic syndrome is diagnosed,appropriate treatment should be taken in time,which may improve the survival rate of the patients.