Title: Hepatitis An Infection presenting as Hemophagocytic lymphohistiocyto-sis (HLH): A case report
Abstract: Background and Aim: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder characterized by the uncontrolled activation of immune cells that could occur sporadically or could be acquired genetically. Secondary HLH may be manifested as immunological activation of the immune system after viral infections, malignancies, and rheumatologic diseases. Case Report: 22 years Female, with no addiction history, presented with nausea & anorexia since 2 weeks. The patient had no history of hematemesis/ malena, altered sensorium, skin rashes, or any other prior de-compensation. On admission the patient had AST, ALT levels of 632, 2139 U/L; Total Bilirubin of 5.9 and INR 3.23. The patient came out to be IgM HAV positive. The patient developed high grade fever after 7-8 days of admission with gradually increasing intensity and frequency of fever. The fever was high grade, intermittent, associated with chills & rigors. Procalcitonin levels 0.44. The patient had raised ferritin levels (>2000), raised triglyceride levels with hypofibrinogenemia. The bone marrow biopsy was suggestive of increased histiocytes with significant hemophagocytosis. The patient was started on intravenous dexamethasone 8 mg BD. The patient started to develop uncontrolled hyperglycemia, psychiatric symptoms on 3rd week of therapy. The patient was started on oral cyclosporine with weekly measurements of cyclosporine levels and the dexamethasone was tapered off. Conclusion: Virus associated Hemophagocytic lymphohistiocytosis is a disease of multi-organ dysfunction with varied and non-specific manifestations. HLH should be suspected in context of unexplained high-grade fever in patients with hepatitis with or without organomegaly.