Disseminated tuberculosis presenting as hemophagocytic lymphohistiocytosis in an immunocompetent adult patient: a case report

Abstract

BackgroundHemophagocytic lymphohistiocytosis is a frequently fatal and likely underdiagnosed disease. It is a rare occurrence in adults and usually secondary to an insult such as viral infections, bacterial infections, autoimmune connective tissue disorders, malignancies and immunocompromised states, in contrast to its childhood counterpart, which is due to a genetic defect but may share some of same genetic etiologies. It is characterized by multisystem inflammation due to unregulated proliferation and infiltration of macrophages and CD8 T cells in the bone marrow, which leads to phagocytosis of red blood cells, platelets, lymphocytes and their precursors.Case presentationA 40-year-old Sri Lankan woman presented with a high-grade fever of 2 weeks’ duration and the initial workup, including a thorough clinical examination, and all the investigations, including a septic screen, were normal. On the 18th day of hospital admission, she was found to have yellowish retinal lesions, which were confirmed as choroid tubercles by the consultant eye surgeon. Two days later she became pancytopenic and a bone marrow biopsy confirmed the diagnosis of hemophagocytic lymphohistiocytosis. She was treated with conventional category-1 antituberculous drugs and an initial 2 weeks with high-dose oral dexamethasone. All the choroid tubercles gradually disappeared and she recovered completely without any complications.ConclusionsIn an adult patient with hemophagocytic lymphohistiocytosis, it is pivotal to understand the underlying etiology, as it needs extensive immunosuppression. If this patient had been treated with immunosuppressants without antituberculous medications, it would have been lethal with disseminated or central nervous system tuberculosis. So, in areas where tuberculosis is endemic, if no underlying cause is found, it may be worth considering antituberculous treatment for these patients. Re-evaluation with thorough clinical examination is of utmost importance in any patient with pyrexia of unknown origin as well as in any disease with unusual manifestations.

Granulomatous Tubercular Hepatitis Presenting as Secondary Hemophagocytic Lymphohistiocytosis: A Case Report and Systematic Review of the Literature

“…We further analyzed 18 more cases published since 2014, including our case, along with the previously published cases. [8][9][10][11][12][13][14][15] Our analysis showed that of 82 patients, 58.5% were men, and the median age of presentation was 40 years. The patients presented with a median duration of 35 days, and all had fever (100%), while majority had hepatomegaly (70%), splenomegaly (77.9%), and lymphadenopathy (62.2%) (Table 1).…”

Hemophagocytic Lymphohistiocytosis in Adults with Intraocular Involvement: Clinicopathologic Features of 3 Cases

“…The term familial hemophagocytic reticulosis was then changed to primary or familial HL and subsequently, nongenetic causes were recognized (i.e., secondary HLH) [1,4]. While primary HLH consists of different monogenic alterations that derive from impaired cytotoxicity of natural killer cells (NK) and CD8+ cytotoxic T lymphocytes (CTL), secondary HLH presents as an acquired complication in several contexts such as infections, malignant neoplasms, autoimmune diseases, post-transplantation, or is iatrogenically induced (Table 1) [8,9,10,11,12,13,14,15]. Regardless of the etiology, HLH is characterized by an uncontrolled activation of NK/CTL that provokes the release of large amounts of proinflammatory cytokines such as IFN-γ, TNF-α, GM-CSF, M-CSF, and IL-2, resulting in hyperstimulation and systemic infiltration by macrophages which, in turn, phagocytose blood cells, mostly red blood cell precursors, and secrete other cytokines responsible for myelosuppression, endothelial damage with coagulopathy, tissue injury, and NK/CTL incessant activation (IL-1, IL-6, and TNF-α) (Fig.…”

A Case of Hemophagocytic Lymphohistiocytosis Triggered by Disseminated Tuberculosis and Hairy Cell Leukaemia after SARS-CoV2 Infection

“…Mycobacterium tuberculosis infection is a known rare trigger of sHLH [13] in immunocompromised patients. The man's occupation, his prolonged steroid therapy and HCL were all significant risk factors for MTB infection, which developed atypically without pulmonary lesions and without detection of MTB-DNA on bronchial wash fluids.…”