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Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disorder marked by excessive immune system activation. Diagnosing HLH is particularly difficult due to its diverse etiology and similar clinical presentation. Patients with HLH often require intensive critical care. The condition is divided into HLH syndrome (genetic defects) and HLH disease, and both can be triggered by factors such as infections, autoimmune diseases, or malignancies. We present here the first documented case of acute HLH secondary to tularemia with septic shock in a 62-year-old female. Initially suspected due to malignancy-induced acute HLH, she was treated with etoposide and dexamethasone and was immediately transitioned to antimicrobials once she was confirmed positive for
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Brahmbhatt et al. (2024) studied this question.
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