Characteristics and prognostic value of pleural effusion in secondary hemophagocytic lymphohistiocytosis

The clinical features of patients with secondary hemophagocytic lymphohistiocytosis (sHLH) complicated with pleural effusion have rarely been evaluated. We retrospectively analyzed 203 patients newly diagnosed with sHLH from July 2015 to July 2019 according to the HLH-2004 protocol. Baseline characteristics, laboratory results, and imaging were reviewed. Pleural effusion was found in 58.6% of the studied sHLH population, and characteristic imaging findings were minimal volume and bilaterality. Patients with pleural effusion had lower PLT counts, HB levels and ALB levels as well as higher sCD25 levels than those without pleural effusion (all p values < 0.05). Multivariate analyses showed that lg(sCD25) and PLT ≤ 65 × 109/L were significant risk factors for developing pleural effusion in sHLH. Regarding prognostic value, survival analysis showed a lower survival probability for patients with pleural effusion than for those without pleural effusion (median OS, 90 vs. 164 days, p = 0.028). In multivariate analysis, pleural effusion was an independent prognostic factor for overall survival (OS) (HR 2.68; 95% CI 1.18–6.11, p = 0.019). Pleural effusion is frequently found in patients with sHLH and is associated with greater inflammation and worse outcomes.