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Hemophagocytic syndrome (HPS) is a life-threatening clinical syndrome that has various presentations, shows rapid progression and is associated with a high mortality. Clinical reports about pulmonary tuberculosis combined with respiratory failure accompanied by HPS are rare.HPS has no special clinical manifestations, and the main presentations include persistent fever, hepatosplenomegaly, hematocytopenia, and rash. In the Intensive Care Unit (ICU), the clinical manifestations of severe infection and secondary HPS overlap, thus there is often a delay in the diagnosis and treatment of HPS.HPS is not an independent disease but represents an excessive inflammatory response due to immune dysfunction induced by various causes such as infection and tumor.The 2 cases in this report show that tuberculosis-associated hemophagocytic syndrome is not easy to find, especially in ICU. There are few clinical reports of pulmonary tuberculosis combined with respiratory failure and HPS. Here, we describe 2 such clinical cases and review the relevant literature in order to deepen our understanding of this disease.