When the usual is unusual – An uncommon presentation of enteric fever

Typhoid fever has an estimated worldwide prevalence of 12–33 million cases with high incidence in India, especially in urban centres. Hemophagocytic lymphohistiocytosis is a rare complication of typhoid fever, which may be fatal if untreated. We describe the case of a young female, with a history of travel from Bali, who presented to our tertiary care centre with high-grade fever, body pain, abdominal pain, loose stools and acute-onset hearing loss. Investigations showed pancytopenia, hyperferritinemia, hypertriglyceridaemia and bone marrow biopsy revealed erythrophagocytic histiocytes and granulomas. Bone marrow culture grew multidrug-resistant Salmonella Typhi. After ruling out other possibilities, including underlying immunodeficiency autoimmune conditions, the diagnosis of secondary HLH was made as per the HLH 2004 diagnostic criteria. The patient responded to culture-sensitive antibiotics, steroids and supportive care. We discuss this case to create awareness about an uncommon complication of a common disease such as typhoid fever.