Clinical features and prognostic factors of adult patients with hemophagocytic lymphohistiocytosis: a retrospective study of 130 adult patients

Abstract

Background Hemophagocytic lymphohistiocytosis (HLH) is a syndrome with high mortality rate that is becoming increasingly common in adults in recent years. The mechanism of HLH in adults is poorly understood. This single-center study focused on the clinical features and prognostic factors of adult HLH patients. Methods We reviewed the medical records of adult HLH patients (age≥15 years) diagnosed and treated at our institution from 2013 to 2022. Patients were followed from index date of prescription until death, or until August 31, 2022. Cox regression analysis was used to estimate hazard ratios (HR) and 95% confidence intervals (CI). Results 130 (78 men, median age, 46 years) satisfied the HLH-2004 diagnostic criteria and were included in the study. 57 (43.8%) patients were malignancy-associated HLH. The median OS of the cohort was 61 days (95% CI 14–108). According to a multivariate Cox retrospective analysis, malignancy-associated HLH (HR=2.421, 95% CI 1.107–2.631, P=0.016) predicted a poorer outcome. Lower platelet count was related with both 30-day mortality (HR=0.987, 95% CI 0.976–0.998, P=0.018) and overall mortality (HR=0.993, 95% CI 0.987–0.999, P=0.019). The adoption of etoposide-related regimens reduced 30-day mortality (HR=0.453, 95% CI 0.252–0.815, P=0.008) and was strongly correlated with a better outcome (HR=0.629, 95% CI 0.407–0.972, P=0.037). Conclusion Our study demonstrated that early diagnosis of HLH and identification of the underlying causes are crucial, low platelet count predicts poor outcome, and that therapy with etoposide dramatically improved prognosis.