Salmonella Paratyphoid Induced Hemophagocytic Lymphohistiocytosis: A Case Report and Literature Review

Abstract

Background: Haemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening syndrome characterized by an excessive inflammatory response. Limited data exist on adult HLH. Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a condition in which immune hyperactivation and deregulation causes hemophagocytosis and organ disorder due to activated histiocytes and macrophages. Symptoms include fever, splenomegaly, abdominal distension and hepatomegaly. Case Report: We report the case of a forty year old man who presented to our Hospital with fever and loose stools and upon investigation had a pancytopenia with hypertriglyceridemia, hepatosplenomegaly, these clinical and laboratory findings raised suspicion of HLH secondary to typhoid fever. 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. In this national, retrospective cohort study, we analysed data from the Pub Med database collected between October 1, 2006 and December 31, 2023 in making of this literature review. Results and Conclusion: Typhoid fever caused by the bacterium salmonella typhi and paratyphi is a serious febrile illness and is more common in the developing countries As a first line intervention, treating the etiology of HLH would be an efficient way to restrict the disease's progression. We report a rare and unique case of secondary HLH due to typhoid fever, this complication is often missed and a prompt diagnosis is essential for the treatment of this fatal disease. It can be primary (familial) or secondary (acquired). Clinically diagnoses can be difficult because of similarities in signs and symptoms with other illnesses as it generally presents with fever, loose stools, and decreased appetite and on Investigation one can expect pan or bi cytopenias, hepatosplenomegaly, hypertriglyceridemia, hypofibrinogenemia. Timely diagnosis of HLH makes it very treatable in majority of cases.