Tuberculosis Presenting as Acute Sepsis and Secondary Hemophagocytic Lymphohistiocytosis
  • Patrícia Campos
    Intensive Care Unit, Centro Hospitalar Universitário do Porto, Porto, Portugal
  • Diana Mano
    Intensive Care Unit, Centro Hospitalar Universitário do Porto, Porto, Portugal
  • Rui Antunes
    Intensive Care Unit, Centro Hospitalar Universitário do Porto, Porto, Portugal


Hemophagocytic lymphohistiocytosis, disseminated tuberculosis, immunosuppressive therapy, aspergillosis, intensive care


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.



  • Shah M, Reed C. Complications of tuberculosis. Curr Opin Infect Dis 2014;27(5):403–410.
  • Narasimhan P, Wood J, Macintyre CR, Mathai D. Risk factors for tuberculosis. Pulm Med 2013;2013:828939.
  • Bridges DA, Bedimo RG. Severe tuberculosis sepsis in an immunocompetent patient. Am J Med 2006;119(3):e11–e14.
  • Kethireddy S, Light RB, Mirzanejad Y, Maki D, Arabi Y, Lapinsky S, et al. Mycobacterium tuberculosis septic shock. Chest 2013;144(2):474–482.
  • Kim YR, Kim DY. Current status of the diagnosis and treatment of hemophagocytic lymphohistiocytosis in adults. Blood Res 2021;56(S1):S17–S25.
  • Lerolle N, Laanani M, Galicier L, Rivière S, Meynard JL, Azoulay E, et al. Factors associated with tuberculosis-associated haemophagocytic syndrome: a multicentre case-control study. Int J Tuberc Lung Dis 2020;24(1):124–130.
  • Hui YMT, Pillinger T, Luqmani A, Cooper N. Haemophagocytic lymphohistiocytosis associated with Mycobacterium tuberculosis infection. BMJ Case Rep 2015;2015:bcr2014208220.
  • Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007;48(2):124–131.
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    Published: 2022-02-15
    Issue: 2022: Vol 9 No 2 (view)

    How to cite:
    Campos P, Mano D, Antunes R. Tuberculosis Presenting as Acute Sepsis and Secondary Hemophagocytic Lymphohistiocytosis. EJCRIM 2022;9 doi:10.12890/2022_003121.

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