1015. Hemophagocytic Lymphohistiocytosis: A Contemporary Single Center Case Series

Abstract

Abstract Background Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive inflammation due lack of normal downregulation of activated macrophages and lymphocytes. We describe clinical and laboratory features, underlying conditions, prognostic factors and outcomes of a contemporary cohort of adults with HLH. Methods We performed a retrospective case series of adults diagnosed and treated for HLH at the University of Kansas Medical Center from January 1, 2006 through September 30, 2017. Patients were identified by ICD9/10 search of our HERON database, followed by manual review of electronic medical records using the HLH-04 criteria for inclusion. Patients were followed until February 20, 2018. Descriptive statistics and Cox survival model for mortality were used for data analyses. Results Among 76 patients reviewed, 39 met the HLH-04 criteria and were included in the cohort. Median age was 46 years , 26 (66.7%) were male, 26 (66.7%) were white, 5 (12.8%) were Asian, and 5 (12.8%) were African American. Twenty-one patients (53.8%) had HLH associated with an infection and 11 patients (28.2%) had HLH associated with malignancy. Median follow up time was 180 days (range 1 – 4002 days). Twenty patients died with case fatality rate of 51.3%, and among those who died, median survival was 17 days (range 1 –180 days). On univariate analysis, variables associated with mortality were: male gender (hazard ratio (HR) 5.32, 95% confidence interval (CI) 1.51-18.78, p=0.004), age ≥ 65 years (HR 2.51, 95% CI 0.99-6.39, p-value 0.005) and bilirubin ≥ 5 mg/dL at diagnosis (HR 3.27, 95% CI 1.34-8.00, p=0.006). These remained significantly associated with mortality on multivariate analysis: male gender (HR 12.34, 95% CI 1.87-29.73, p=0.020) and age ≥ 65 years (HR 7.45, 95% CI 1.87-29.73, p=0.008) and bilirubin ≥ 5 mg/dL (HR 4.75, 95% CI 1.09-20.72, p=0.002). Conclusion In this retrospective case series of adults with HLH, mortality at a median follow up of 180 days remained high (53%) and is associated with older age age ( >65 years), male gender, and elevated bilirubin ≥ 5mg/dL at diagnosis. Better therapeutic options for this syndrome are needed. Disclosures All Authors: No reported disclosures