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Purpose: We present de case of a 27-year-old woman admitted to ICU after scheduled splenectomy to study her short course of fever, leukopenia and splenic space-occupying lesions and splenomegaly. She has been previously treated with Infliximab due to indeterminate colitis and completed correct tuberculosis prophylaxis., Materials and Methods: We reviewed our case in our regional Electronic Health DataBase IANUS and compared it with other case reports in literature, found in PubMed, with keywords tuberculosis, inflammatory disease and hemophagocytic lymphohistiocytosis., Results: After splenectomy, she needed intensive care due to acute respiratory failure, alveolar-interstitial pulmonary infiltrates, right pleural effusion and fever. Bone marrow aspirate resulted in hemophagocytic lymphohistiocytosis. Only multidisciplinary management in ICU and combined treatment with chemotherapy for hemophagocytic syndrome and tuberculostatics achieved her curation., Conclusion: Tuberculosis must always be considered in the differential diagnosis of hemophagocytic lymphohistiocytosis and acute respiratory failure despite correct prior prophylaxis.