Secondary Hemophagocytic Lymphohistiocytosis Due to Typhoid Fever

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal hyper-inflammatory state that is caused by a highly activated but ineffective immune system. It can be primary or secondary to triggers like infections, malignancies, and autoimmune conditions. The authors present the case of a young male with a fever and abdominal pain due to typhoid. He continued to have a high-spiking fever and developed dyspnea, requiring oxygen therapy despite being treated with appropriate antibiotics. Laboratory evaluation revealed cytopenias and deranged liver function tests, and abdominal imaging revealed hepatosplenomegaly. These clinical and laboratory findings raised suspicion of HLH secondary to typhoid fever. Further investigations were suggestive of hyperferritinemia and hypofibrinogenemia, and bone marrow aspirates showed hemophagocytes. The patient was treated with immunosuppression (dexamethasone) and antibiotics and showed remarkable recovery. Hemophagocytic lymphohistiocytosis should be suspected in patients with tropical infections like enteric fever, tuberculosis, malaria, dengue, etc. that worsen despite appropriate treatment, as late diagnosis is associated with greater mortality.