Hemophagocytic lymphohistiocytosis (HL) is a rare syndrome characterized by a hyperinflammatory state, resulting from an excessive but ineffective immune response. There is a continuous stimulation of TCD8 + lymphocytes, associated with an uncontrolled release of cytokines, causing the infiltration of multiple organs by histiocytes and activated lymphocytes. HL can be a primary condition as a consequence of genetic disorder that most often affects children, or it can be secondary to neoplasms, autoimmune conditions or various infectious diseases in patients of all ages. HL caused by infection by Mycobacterium tuberculosis is highly unusual, with few cases reported in the literature. There is no clinical manifestation or laboratorial finding that is specific to HL, and a high index of clinical suspicion is necessary for the correct diagnosis, which is usually confirmed by biopsy. Treatment consists of controlling the causative event and the use of immunosuppressant drugs such as corticosteroids, etoposide, and cyclosporine to suppress the exacerbated immune response. We report the case of a patient who developed HL 2 months after liver transplantation. The initial presentation was persistent fever, prompting a search for a site of infection and the use of broad-spectrum antibiotics. As the clinical condition of the patient continued to deteriorate, HL was diagnosed through a bone marrow biopsy, and a cerebrospinal fluid culture positive for M. tuberculosis established the diagnosis of disseminated tuberculosis. Despite optimal treatment with immunosuppressors and antituberculosis drugs, there was no significant response and the patient died. This article is compliant with the Helsinki Congress and the Istanbul Declaration.
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