Clinical Features And Outcomes Of 205 Patients With Secondary Adult Hemophagocytic Lymphohistiocytosis:A Retrospective Study From A Single Center

Background:Hemophagocytic lymphohistiocytosis(HLH)is a rare clinical syndrome characterized by an infrequent but immune-mediated life-threatening disease.The diagnosis of HLH in adults is challenging due to its occurring in many disease entities.Methods:This study enrolled 205 patients met the HLH-2004 diagnostic criteria.We analyzed the clinical and laboratory findings and prognostic factors of adult HLH in a large cohort managed at a single medical center from January 2011 to December 2015.Results:The median age was 52 years(range,18-90 years)and 123(60.0%)were male.Over 95%patients manifested fever,hyperferritinemia,and elevated lactate dehydrogenase.Underlying causes of HLH were as follows:119(58.0%)malignancies,83(40.5%)infections,14(6.8%)unknown causes and 14(6.8%)autoimmune disorders.The median overall survival was 55 days.And elderly patients(age>60 years)had a markedly worse survival compared with young patients(age<60 years)(median overall survival 24 days vs.159 days,respectively;P<0.001).In a multivariable analysis,platelet count<40×109/L(HR = 2.273;95%CI:1.246-5.948;P = 0.012),prothrombin time prolonged>3 seconds(HR = 2.008;95%CI:1.190-3.390;P = 0.009),adenosine deaminase(ADA)>89 U/L(HR = 1.706;95%CI:1.015-2.865;P = 0.044)and low density lipoprotein(LDL)>3.29 mmol/L(HR = 0.196;95%CI:0.048-0.806;P = 0.024)were correlated with poor survival.Conclusion:Therefore it is of great importance to improve our understanding of this syndrome,validate diagnostic criteria,and help develop effective treatment strategies.