Hepatitis A infection related haemophagocytic syndrome: a case report and systematic review

Abstract

Haemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome of excessive inflammation and tissue destruction owing to abnormal immune activation. We report an unusual case of haemophagocytosis associated with hepatitis A virus (HAV) infection in a 21-year-old man. This was further complicated by haemolysis secondary to G-6-PD deficiency and fungal sepsis. Our patient was treated successfully with intravenous immunoglobulin (IVIg) and supportive care. A systematic review of all reported cases of HAV associated haemophagocytosis is presented.

Macrophage Activation Syndrome in Viral Sepsis

“…Moreover, HLH can result from infection with hepatitis viruses, with the majority of cases involving HAV and, less commonly, HBV or HCV infection [92][93][94]. Two systematic reviews of HAV-associated HLH cases have identified hepatomegaly and splenomegaly as distinguishing features of HAV-HLH, as these signs are rarely observed in patients with HAV infection who do not develop HLH [63,95]. Also, they noted that thrombocytopenia could be an early sign of HAV-HLH [63,95].…”

“…Two systematic reviews of HAV-associated HLH cases have identified hepatomegaly and splenomegaly as distinguishing features of HAV-HLH, as these signs are rarely observed in patients with HAV infection who do not develop HLH [63,95]. Also, they noted that thrombocytopenia could be an early sign of HAV-HLH [63,95]. However, while hyperferritinemia can occur solely due to HAV infection, higher levels may indicate the presence of HLH [63].…”

“…Also, they noted that thrombocytopenia could be an early sign of HAV-HLH [63,95]. However, while hyperferritinemia can occur solely due to HAV infection, higher levels may indicate the presence of HLH [63].…”