Hemophagocytic Lymphohistiocytosis: A Rare Complication of Acute Hepatitis E Infection

Nihar Desai1, Darpan Vetal2, Abhishek Kulkarni2

  • 1Assistant Professor, Department of General Medicine, Lokmanya Tilak Municipal Medical College and General Hospital (LTMGH), Mumbai, Maharashtra, India, Corresponding Author.

Hemophagocytic lymphohistiocytosis (HLH) is an aggressive hematological disorder caused by uncontrolled activation of cytotoxic T-cells (CTL), natural killer (NK) cells, and macrophages leading to hyperinflammation and cytokine storm. The clinical course is characterized by high-grade fever, cytopenia, and multiorgan dysfunction. HLH is classified as either primary/familial or secondary, the latter being most often triggered by infections, malignancies, and autoimmune disorders. Viral infections are commonly known to cause HLH with Epstein-Barr virus (EBV), cytomegalovirus (CMV), influenza virus, adenovirus, and parvovirus being most often implicated. Hepatitis E virus (HEV) has infrequently been reported to cause HLH with less than five cases being reported in the literature. We report a case of a young man who presented with hepatitis E-associated HLH.