Background:
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome frequently complicated by circulatory collapse and multiorgan failure. Although septic shock (SS) is a recognized predictor of poor outcomes in HLH, the clinical significance of cardiogenic shock (CS) in this population remains poorly defined.
Methods:
Using the National Inpatient Sample (NIS) database (2016–2020), we identified 2,506 adult hospitalizations with a diagnosis of HLH and stratified patients into three cohorts: no shock (NS; n = 2,015), SS (n = 481), and CS (n = 10). Primary outcomes included in-hospital mortality, cardiac arrest, acute kidney injury (AKI), dialysis initiation, acute respiratory failure (ARF), and mechanical circulatory support (MCS) utilization. Secondary outcomes included length of stay (LOS) and total hospitalization charges. Multivariable logistic regression models were adjusted for demographics and baseline comorbidities
Results:
Patients with CS had a significantly greater burden of cardiovascular comorbidity, including heart failure, coronary artery disease, chronic kidney disease, and chronic obstructive pulmonary disease (all p < 0.001 vs. NS). In-hospital mortality was highest in the CS cohort (70.0%), followed by SS (56.7%) and NS (14.1%) (p < 0.001). Both shock phenotypes were associated with increased rates of cardiac arrest, AKI, dialysis initiation, and ARF compared with NS. MCS utilization was markedly higher in CS than in SS (20.0% vs. 1.7%, p < 0.001). After multivariable adjustment, SS independently predicted mortality (adjusted odds ratio [aOR] 8.25; 95% confidence interval [CI] 6.49–10.48), AKI (aOR 7.45; 95% CI 5.80–9.57), dialysisinitiation (aOR 5.29; 95% CI 4.03–6.94), ARF (aOR 5.87; 95% CI 4.70–7.34), and cardiac arrest (aOR 4.02; 95% CI 2.65–6.12) (all p ≤ 0.001). CS independently predicted mortality (aOR 2.91;95% CI 1.25–6.81; p = 0.014), ARF (aOR 3.17; 95% CI 1.32–7.63; p = 0.010), and MCSutilization (aOR 22.58; 95% CI 2.78–183.60; p = 0.004).
Conclusion:
Shock complicating HLH is associated with substantially worse clinical outcomes. SS confers the greatest burden of multiorgan dysfunction, whereas CS, though rare, identifies a distinct high-risk cardiovascular phenotype characterized by extensive cardiac comorbidity, elevated mortality, and markedly greater need for advanced hemodynamic support.
Keywords: Hemophagocytic Lymphohistiocytosis; Cardiogenic Shock; Septic Shock; Mechanical Circulatory Support; Outcomes
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