The prognostic value of liver function abnormalities in adult secondary hemophagocytic lymphohistiocytosis.

BACKGROUND: Adult secondary hemophagocytic lymphohistiocytosis (sHLH) is a disorder of immune dysregulation and hyperinflammation that is frequently accompanied by hepatic dysfunction. Evidence describing liver function test (LFT) abnormalities and their prognostic associations in adults with sHLH remains limited. METHODS: In this retrospective study, clinical data and LFT results were obtained at admission from 269 patients with sHLH. Multivariable logistic regression and restricted cubic spline models were used to evaluate the associations between LFTs and in-hospital mortality. RESULTS: Abnormal alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase (ALP), and total bilirubin (TBIL) levels were present in 56.5, 75.8, 61.3, and 42.0% of patients, respectively. In multivariable models, higher log-transformed ALP was associated with 28-day mortality (OR 2.84, 95% CI 1.16-7.22; P = 0.025), and higher log-transformed TBIL was associated with 7-day mortality (OR 2.77, 95% CI 1.06-7.42; P = 0.038) and 28-day mortality (OR 4.84, 95% CI 2.35-10.56; P < 0.001). When analyzed as categorical variables, abnormal TBIL was associated with greater odds of 7-day (OR 3.56, 95% CI 1.25-11.27; P = 0.022) and 28-day mortality (OR 2.03, 95% CI 1.12-3.71; P = 0.020), whereas abnormal ALP was associated with 28-day mortality (OR 2.34, 95% CI 1.26-4.48; P = 0.008). Restricted cubic spline analysis showed a non-linear association between ALP and 28-day mortality (P for non-linearity = 0.018). Neither ALT nor AST was associated with either outcome after multivariable adjustment. CONCLUSION: Abnormal LFTs were common at admission, and ALP and TBIL may be useful prognostic markers of short-term in-hospital mortality in adults with sHLH.