1015. Hemophagocytic Lymphohistiocytosis: A Contemporary Single Center Case Series. (4th December 2021)

• Record Type: Journal Article
• Authors: Jabr, Ra'ed; Ryan, Miriam; Mohyuddin, Ghulam Rehman; Hammoud, Kassem; Male, Heather; El Atrouni, Wissam
• Is Part Of: Open forum infectious diseases. Volume 8(2021)Supplement 1
• Page Start: S598
• Page End: S599
• Publication Date: 2021-12-04
• Subjects: Communicable diseases -- Periodicals; Medical microbiology -- Periodicals; Infection -- Periodicals; 616.9
• DOI: 10.1093/ofid/ofab466.1209
• Languages: English
• ISSNs: 2328-8957

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