Hemophagocytic Lymphohistiocytosis-A Rare Complication of Hepatitis A Virus Infection

Abstract

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a heterogeneous group of clinical syndromes characterized by activation and subsequent uncontrolled non-malignant proliferation of T-lymphocytes, histiocytes and macrophages leading to a cytokine storm and manifesting as prolonged fever, organomegaly, cytopenia, hyperferritinemia and demonstrable hemophagocytosis in the bone marrow. Case Presentation: Here we present a case of infection associated HLH in a five-year-old girl as a complication of Hepatitis A virus infection. The girl developed acute fulminant hepatic failure with multisystem involvement and was treated with a short course of steroid. Conclusions: Hemophagocytic lymphohistiocytosis is broadly classified as two types, either familial or acquired. The acquired variety may be secondary to any type of infection, mostly viral. Hepatitis A virus is rarely reported to give rise to HLH in the pediatric population. Less intensive immunosuppressive therapy with steroids was successful in maintaining remission.