Tuberculosis Presenting as Acute Sepsis and Secondary Hemophagocytic Lymphohistiocytosis

We report the case of a 61-year-old man admitted to our emergency department with fever. At admission, he was hypotensive and tachycardic. In the initial investigation, elevation of inflammatory parameters, acute kidney injury (Kidney Disease Improving Global Outcomes (KDIGO) 3), hyperbilirubinemia, and hepatic cytocholestasis were evident. Empirical antibiotic therapy was started, after sepsis was assumed without an identifiable cause. His condition took an unfavorable clinical course, with respiratory failure, hepatosplenomegaly, pancytopenia, hyperferritinemia and hypofibrinogenemia. Microbial culture studies and a general immunological study were negative and lymphoproliferative disease was therefore excluded. Bone marrow aspirate revealed hemophagocytosis without granulomas. A diagnosis of hemophagocytic lymphohistiocytosis was assumed and pulse methylprednisolone therapy initiated. As this resulted in only a transient improvement, immunoglobulin and rituximab were initiated as a second-line therapy. The patient sadly had an unfavorable outcome despite all measures undertaken. In the postmortem study, Mycobacterium tuberculosis complex was isolated in the bone marrow aspirate, which led to the postmortem diagnosis of disseminated tuberculosis and angioinvasive pulmonary aspergillosis. The clinical presentation of disseminated tuberculosis is non-specific and hemophagocytic lymphohistiocytosis is one of its rare presentations. The mortality rate of hemophagocytic lymphohistiocytosis is high and increases with delayed diagnosis of the underlying condition and respective treatment. LEARNING POINTS: Hemophagocytic lymphohistiocytosis should be considered in patients presenting with fever, lymphadenopathy, splenomegaly, cytopenias, hyperferritinaemia and hypertriglyceridemia. Despite its rarity, tuberculosis should be considered as an etiology of hemophagocytic lymphohistiocytosis and, if suspected, antituberculosis therapy should be initiated early, even in the absence of a definite diagnosis. Immunosuppressant therapy increases the risk of opportunistic infections, which establishes the need for prophylactic antibiotic, antifungal, and antiviral drugs.