Secondary hemophagocytic lymphohistiocytosis mimicking sepsis in hepatitis A-E coinfection: spontaneous recovery with supportive care.

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome caused by dysregulated immune activation. Viral infections, particularly Epstein-Barr virus, are well-known triggers; however, HLH associated with acute viral hepatitis is rare, especially in the setting of hepatitis A (HAV) and hepatitis E (HEV) coinfection. A 30-year-old man presented with fever following the onset of jaundice, along with fatigue and dyspnea. Laboratory evaluation revealed severe hyperbilirubinemia, markedly elevated transaminases, anemia, hyperferritinemia, and hypertriglyceridemia. He also had hemolysis due to glucose 6 phosphate dehydrogenase(G6PD) deficiency. Bone marrow examination demonstrated hemophagocytosis. Viral m arkers confirmed HAV and HEV coinfection. Although he fulfilled five out of eight HLH-2004 criteria, sepsis markers like raised procalcitonin and leukocytosis posed diagnostic challenge. He was managed with supportive care alone, and showed spontaneous clinical and biochemical improvement without immunosuppressive therapy. This case highlights a probable HLH spectrum disorder triggered by HAV-HEV coinfection. It underscores the importance of considering HLH in patients with acute viral hepatitis who develop persistent fever and cytopenia, while also recognizing that selected patients may improve with supportive care alone.