Vol. 7, Issue 2, Part C (2025)

Background: Typhoid fever, caused by Salmonella typhi, continues to be a major cause of morbidity and mortality in India. Atypical or unusual manifestations of it may closely resemble other infectious, hematological or autoimmune disorders, complicating timely diagnosis. Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal hyper-inflammatory syndrome characterized by persistent fever, cytopenias, hepatosplenomegaly, markedly elevated ferritin. Secondary HLH triggered by typhoid fever is rare. When accompanied by chronic granulomatous inflammation, it may mimic malignancy or tuberculosis, further adding to the diagnostic complexity. We report a case of typhoid fever presenting with secondary HLH and chronic granulomatous inflammation.

Case Presentation: A 13-year-old boy presented in our department with 14 days of high-grade intermittent fever, generalized body pains, watery stools and intermittent epistaxis. Laboratory evaluation revealed anemia, leukopenia, thrombocytopenia, elevated transaminases, high CRP, markedly raised ferritin, hypertriglyceridemia and low fibrinogen, fulfilling HLH-2004 diagnostic criteria. Infectious workup for dengue, leptospirosis, scrub typhus and chikungunya was negative. Bone marrow aspiration showed hemophagocytosis and biopsy demonstrated chronic granulomatous inflammation. Bone marrow culture grew Salmonella typhi, confirming typhoid-associated secondary HLH. The patient was treated with intravenous steroids and culture-directed antibiotics, resulting in gradual clinical improvement and normalization of hematologic and biochemical abnormalities. He was discharged in stable condition on tapering steroids and oral antibiotics.

Conclusion: Typhoid fever associated HLH is uncommon but should be considered in children with persistent fever and cytopenias. Bone marrow culture is invaluable when blood cultures are negative. Timely diagnosis and combined antimicrobial and immunosuppressive therapy are essential in achieving favorable outcomes.