Key Points Neutralization of IFN-γ with emapalumab can reverse severe, refractory hemophagocytic lymphohistiocytosis. Neutralizing IFN-γ did not impair control of multiple viral and other infections in a severely ill patient.

Early mortality remains a major challenge for the treatment of hemophagocytic lymphohistiocytosis (HLH), which warrants the need for prompt risk stratification in the early phase of the disease. We retrospectively analyzed clinical features of a cohort of pediatric patients managed at a tertiary hospital in southern China from 2005 to 2015. A total of 116 patients (median age 27.5 months) with predominantly secondary HLH were included. In a multivariate Cox regression model, neutrophils <0.5 × 10(9)/L (risk ratio (RR) = 5.01; 95 % confidence interval (CI) 1.55-16.20; P = 0.007), total bilirubin over twofold upper limit of normal value (RR = 2.86; 95 % CI 0.83-9.88; P = 0.097), and albumin ≤20 g/L (RR = 5.79; 95 % CI 1.70-19.73; P = 0.005) at diagnosis were independent risk factors for 30-day mortality. The 30-day overall survival rate (OS) of patients with three risk factors was significantly lower than that of patients with zero to two risk factors (0 vs 90.7 %; P<0.001). Patients with three risk factors were 64-fold more likely to have early adverse outcome as compared to patients with zero to two risk factors (RR = 64.45; 95 % CI 18.35-226.33; P<0.001). Platelet count normalization in 2 weeks was an independent predictor for resolution after initial therapy with an odds ratio (OR) of 18.4 (95 % CI 2.7-122.9; P = 0.003). Our results indicate that severe neutropenia and liver function damage are prognostic factors for early death in HLH and platelet count normalization in 2 weeks is a critical predictor for resolution after initial therapy.

It was the objective of this work to systematically evaluate the role of vitamin E supplementation in the prevention of stroke. Eligible studies were identified from Medline, Embase and Cochrane Library. The efficacy data is the relative risk (RR) for the events of stroke. Thirteen randomised controlled trials (RCTs), with 166,282 participants in total, were analysed. The pooled results showed no significant benefit in the vitamin E group with respect to stroke of any type (RR 1.01; 95% confidence interval [CI]: 0.96, 1.07); ischaemic stroke (RR 1.01; 95% CI: 0.94, 1.09), haemorrhagic stroke (RR 1.12; 95% CI: 0.94, 1.33), fatal stroke (RR 0.94; 95% CI: 0.77, 1.14), and non-fatal stroke (RR 0.99; 95% CI: 0.91, 1.08). Administration of vitamin E 300 IU/day or more also gain no benefit (RR 0.99; 95% CI: 0.92, 1.06), as well as vitamin E less than 300 IU (RR 1.05; 95% CI: 0.96, 1.15). Vitamin E supplementation gained benefit of preventing stroke for neither healthy people (0.92; 0.83, 1.03) nor others at high risks in baseline (RR 1.05; 95% CI: 0.98, 1.12). Administration of synthetic vitamin E gain no benefit (RR 1.02; 95% CI: 0.96, 1.09), as well as the natural source vitamin E (RR 0.99; 95% CI: 0.89, 1.09). In conclusion, there is a lack of statistically significant or clinically important benefit of vitamin E supplementation in the prevention of stroke.