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The intensive care unit (ICU) admission of patients with hematologic malignancies is gradually increasing. Life-threatening events are common, and acute respiratory distress syndrome (ARDS) is one of the most critical conditions. The aim of this study was to investigate the clinical characteristics and outcomes of ARDS in patients with hematological malignancies admitted to the ICU.A retrospective study was performed on all patients with ARDS with hematological malignancies in a single tertiary teaching hospital between 2008 and 2015. Data on the treatment of and the outcomes of ARDS were collected to determine the clinical characteristics associated with ICU mortality.During the 8-year study period, among a total of 821 patients with ARDS admitted to the ICU, all 185 patients with hematological malignancies were included in the analysis. Most of the patients (88.1%) had moderate-to-severe ARDS, and the median PaO2/FiO2 ratio was 122 (interquartile range: 88-157). The overall ICU mortality rate was 57.3% (50.0% for mild, 52.0% for moderate, and 67.7% for severe ARDS). After the univariate and the multivariate logistic regressions, the factors independently associated with a higher ICU mortality were severe ARDS (odds ratio [OR]: 2.47; 95% confidence interval [CI]: 1.17-5.25), identification of carbapenem-resistant gram-negative bacteria (OR: 6.61; 95% CI: 1.31-33.41), the amount of blood product transfusion (OR: 1.25; 95% CI: 1.13-1.38), and the progressive or refractory disease (OR: 3.01; 95% CI: 1.31-6.91). Mortality was independently lower in patients who received the initial low tidal volume ventilation (OR: 0.37, 95% CI: 0.14-0.96).The outcome of ARDS in patients with hematological malignancies is associated with the severity of the underlying diseases, the presence of multidrug-resistance pathogens, and the amount of transfusion; however, strict application of low tidal volume ventilation may improve the outcome of these patients at the time of diagnosis.