Liver function tests in patients with secondary hemophagocytic lymphohistiocytosis: prevalence and impact on in-hospital mortality

Abstract

Abstract Adult secondary hemophagocytic lymphohistiocytosis (sHLHa) is a condition of immune dysregulation and hyperinflammation that is commonly associated with hepatic dysfunction. However, to date, no studies have systematically described liver test abnormalities in patients with sHLHa. The contribution of abnormalities in liver function tests (LFTs) to the mortality in patients with sHLHa remains unclear. In this retrospective study, clinical records and LFTs were obtained from 269 patients with sHLHa at admission. Multivariable logistic models and restricted cubic spline models were used to evaluate the relationship between LFTs and in-hospital mortality. Of these sHLH patients, 56.5% had abnormal ALT, 75.8% had abnormal AST, 61.3% had abnormal ALP, and 42.0% had abnormal total bilirubin. Multivariate analysis revealed that abnormal LFTs (as continuous variables) were associated with a higher risk of in-hospital mortality (OR28 − day=2.84, P = 0.025 for ALP; OR7 − day=2.77, P = 0.038 and OR28 − day=4.84, P<0.001 for TBIL). Whereas, as binary categorical variable, compared with that of normal TBIL levels, abnormal TBIL levels were associated with increased 7-day mortality (OR = 3.56, P = 0.022) and 28-day mortality (OR = 2.03, P = 0.020). Abnormal ALP was associated with increased 28-day mortality (OR = 2.34, P = 0.008) but not 7-day mortality. In addition, the restricted cubic spline curve displayed a nonlinear and positive relationship between ALP and 28-day mortality (P for nonlinearity = 0.018). We also found no association between AST or ALT and outcomes. At admission, sHLHa patients commonly have abnormal LFTs. After multivariable adjustment, abnormal ALP and TBIL were independently associated with in-hospital mortality and may represent an important prognostic variable.